This is from the Lupus Foundation of America’s web page. You can read more at www.lfa.org.
What is Lupus
Lupus is a chronic, autoimmune disease that can damage any part of the body (skin, joints, and/or organs inside the body). Chronic means that the signs and symptoms tend to last longer than six weeks and often for many years. In lupus, something goes wrong with your immune system, which is the part of the body that fights off viruses, bacteria, and germs (“foreign invaders,” like the flu). Normally our immune system produces proteins called antibodies that protect the body from these invaders. Autoimmune means your immune system cannot tell the difference between these foreign invaders and your body’s healthy tissues (“auto” means “self”) and creates autoantibodies that attack and destroy healthy tissue. These autoantibodies cause inflammation, pain, and damage in various parts of the body.
- Lupus is also a disease of flares (the symptoms worsen and you feel ill) and remissions (the symptoms improve and you feel better). Lupus can range from mild to life-threatening and should always be treated by a doctor. With good medical care, most people with lupus can lead a full life.
- Lupus is not contagious, not even through sexual contact. You cannot “catch” lupus from someone or “give” lupus to someone.
- Lupus is not like or related to cancer. Cancer is a condition of malignant, abnormal tissues that grow rapidly and spread into surrounding tissues. Lupus is an autoimmune disease, as described above.
- Lupus is not like or related to HIV (Human Immune Deficiency Virus) or AIDS (Acquired Immune Deficiency Syndrome). In HIV or AIDS the immune system is underactive; in lupus, the immune system is overactive.
- Our research estimates that at least 1.5 million Americans have lupus. The actual number may be higher; however, there have been no large-scale studies to show the actual number of people in the U.S. living with lupus.
- It is believed that 5 million people throughout the world have a form of lupus.
- Lupus strikes mostly women of childbearing age (15-44). However, men, children, and teenagers develop lupus, too.
- Women of color are 2-3 times more likely to develop lupus.
- People of all races and ethnic groups can develop lupus.
- More than 16,000 new cases of lupus are reported annually across the country.
To start this post, I find it is important to describe what the definition of tremor is. Here is the definition from wikipedia:
A tremor is an involuntary, somewhat rhythmic, muscle contraction and relaxation involving to and fro movements (oscillations or twitching) of one or more body parts. It is the most common of all involuntary movements and can affect the hands, arms, eyes, face, head, vocal folds, trunk, and legs. Most tremors occur in the hands. In some people, tremor is a symptom of another neurological disorder. A very common kind of tremor is the chattering of teeth, usually induced by cold temperatures or by fear.
This would seem to be a complete definition but the things I experience do not necessarily fit into this tight definition. I do experience hands shaking, sometimes lip quivering, and muscle twitches at times. The shaking I get that drives me bonkers is where it feels like the whole inside of my body is shaking and it may or may not show in my hands or other body area. It is quite frustrating and scary. It makes me stop whatever I am doing and have to try to lay down and rest to relax my body. It does not seem to be anxiety related either. It cans trike me at random and is puzzling and frightening. So, as I usually do, I thought I would research this out too.
Amazingly, I found not one shred of medical information regarding this, other than others who have had this experience. I usually find things on medical boards or places like medline or webmd but not in this instance. It made me wonder if any of you have had this happen to you too.
I know I saw quite a few others asking this same question as well. I know I am not alone in this. It just may take some time until more is known in the realm of medical professionals for me to find anything online.
Part 4 of this series from the Mayo Clinic website.
By Mayo Clinic staff
Peripheral neuropathy risk factors include:
- Diabetes, especially if your sugar levels are poorly controlled
- Alcohol abuse
- Vitamin deficiencies, particularly B vitamins
- Infections, such as Lyme disease, shingles (varicella-zoster), Epstein-Barr, hepatitis C and HIV/AIDS
- Autoimmune diseases, such as rheumatoid arthritis and lupus, in which your immune system attacks your own tissues
- Kidney, liver or thyroid disorders
- Exposure to toxins
- Repetitive physical stress, possibly from occupational activities
This information is from the NINDS website.
NINDS Neurological Sequelae Of Lupus Information Page
Synonym(s): Lupus – Neurological Sequelae, Systemic Lupus Erythematosus
Table of Contents (click to jump to sections)
What are Neurological Sequelae Of Lupus?
Lupus (also called systemic lupus erythematosus) is a disorder of the immune system. Normally, the immune system protects the body against invading infections and cancers. In lupus, the immune system is over-active and produces increased amounts of abnormal antibodies that attack the body’s tissues and organs. Lupus can affect many parts of the body, including the joints, skin, kidneys, lungs, heart, nervous system, and blood vessels. The signs and symptoms of lupus differ from person to person; the disease can range from mild to life threatening.
Initial symptoms of lupus may begin with a fever, vascular headaches, epilepsy, or psychoses. A striking feature of lupus is a butterfly shaped rash over the cheeks. In addition to headache, lupus can cause other neurological disorders, such as mild cognitive dysfunction, organic brain syndrome, peripheral neuropathies, sensory neuropathy, psychological problems (including personality changes, paranoia, mania, and schizophrenia), seizures, transverse myelitis, and paralysis and stroke.
Is there any treatment?
There is no cure for lupus. Treatment is symptomatic. With a combination of medication, rest, exercise, proper nutrition, and stress management, most individuals with lupus can often achieve remission or reduce their symptom levels. Medications used in the treatment of lupus may include aspirin and other nonsteroidal anti-inflammatory medications, antimalarials, corticosteroids, and immunosuppressive drugs.
What is the prognosis?
The prognosis for lupus varies widely depending on the organs involved and the intensity of the inflammatory reaction. The course of lupus is commonly chronic and relapsing, often with long periods of remission. Most individuals with lupus do not develop serious health problems and have a normal lifespan with periodic doctor visits and treatments with various drugs.
What research is being done?
Investigators researching lupus seek to increase scientific understanding of the disorder and to find ways to treat, prevent, and ultimately, cure it. Several components of the National Institutes of Health support research on lupus.
I am posting this link here because it concerns those of us with lupus. However, I would like to add that while this list is good advice for most people with lupus, some of these things may not impact all lupus patients. The disease is known for manifesting itself in a multitude of ways in each person. In other words, what harms one person may not harm another or cause a flare. So, please read this article from Johns Hopkins and discuss it with your doctor. Enjoy!
Things to Avoid
If you have lupus or a condition that predisposes you to lupus, such as undifferentiated connective tissue disease (UCTD), there are certain foods and medications that you should avoid. The substances listed below have shown to induce lupus signs and flares and should be avoided by people with lupus or autoimmune diseases suggesting “pre-lupus.”
People with lupus should avoid the sun, since sunlight can cause rashes and flares. Some people are more sensitive to sunlight than others, but all people with lupus are advised to be cautious when they are outside. Of course, it would be impractical to completely avoid going outdoors, but try to be prepared. Carry a sunscreen with an SPF of at least 70 and be sure that your sunscreen contains Helioplex, an ingredient that blocks UV-A and UV-B rays, both of which are harmful to people with lupus. Apply sunscreen to all areas of the body, even those covered by your clothes, since most normal clothing items only protect your skin to the level of SPF 5. In addition, carry a hat with you when you know you will be outside. Certain sportswear manufacturers now make hats with SPF built into the material, which may be helpful for people with greater photosensitivity.
(2) Bactrim and Septra (sulfamethoxazole and trimethoprim)
Bactrim and Septra are antibiotics that contain sulfamethoxazole and trimethoprim. They are grouped as “sulfa” antibiotics because they contain a substance called sulfonamide. Bactrim and Septra are often prescribed for bacterial infections, especially urinary tract infections. They are also sometimes given prophylactically (i.e., to prevent infection), especially in people taking immunosuppressive medications. However, it is very important that you avoid Bactrim and Septra, because these antibiotics are known to cause an increase in sun sensitivity and lower blood counts in people with lupus, resulting in lupus flares. Several medications can be used instead of Bactim or Septra for the prevention and treatment of infection; perhaps the most frequently used substitute is Dapsone (diaminodiphenyl sulfone) to prevent Pneumocystis pneumonia.
Scientists believe that three substancs in garlic—allicin, ajoene, and thiosulfinates—rev-up your immune system by enhancing the activity of white blood cells, particularly macrophages and lymphocytes. Scientists also believe that the sulfur components of garlic help to prevent and suppress cancer in the body. For this reason, garlic is often used as a supplement to combat colds and infections. Unfortunately, the enhancement of immune response is counterproductive in people with autoimmune disease such as lupus, because their immune system is already overactive. As a result, people with lupus and lupus-like signs should avoid cooking with garlic and adding it to food. Of course, a tiny amount of the herb will not harm you, but try to consciously avoid purchasing and preparing foods with garlic.
(4) Alfalfa Sprouts
Alfalfa sprouts contain an amino acid called L-canavanine that can increase inflammation in people with lupus by stimulating the immune system. As a result, people with lupus and similar autoimmune conditions should avoid alfalfa sprouts completely.
(5) Melatonin and Rozerem (ramelteon)
Melatonin is a hormone secreted by the pineal gland in your brain that regulates other hormones in the body that control how your body reacts to daily patterns of light and dark. Melatonin release is suppressed during the light hours of the day and stimulated by dark, helping you stick to patterns of nighttime sleep and daytime wakefulness. As a result, melatonin is often used as a sleep aid over other medications. Melatonin and melatonin-containing supplements should be avoided in people with lupus and other autoimmune disorders because they may stimulate the immune system. In addition, people with these conditions should also avoid the prescription sleep aid Rozerem (ramelteon), because it mimics melatonin in the body. It is important that you understand the necessity of avoiding both melatonin and Rozerem, since sleep aids are often used to help people with fibromylagia and other conditions to attain normal sleep patterns. In general, be sure that you speak with your physician before taking any new medications or supplements.
Echinacea is often used as a dietary supplement to boost the immune system against colds and other illnesses. However, because Echinacea boosts your immune system, it may cause flares in people with autoimmune diseases such as lupus. In fact, Echinacea supplements sold in Europe bear warning labels that advise against use by people with autoimmune diseases. As a result, people with lupus and other autoimmune diseases should avoid these supplements. In general, it is important that you speak with your physician before taking any new medications or supplements.
I am repeating this post because it is worth repeating this time of year.
This article I found online discusses the issues the summer season can provoke and offers some great tips on getting through summer overall. I know I have discussed how cold affects my lupus, but unless you have lupus, you may not be aware that summer heat and sun can also wreak havoc on bodies that are already off kilter. This information comes from the web :associated content on yahoo. I hope you will read it and use the suggestions it presents for dealing with your lupus or your friend with lupus. It helps to educate others on the very real dangers the lupus patient can experience. Enjoy! I found it enlightening for myself and learned several new things.
Lupus and Heat
SLE or lupus and other autoimmune disorders often have higher incidences of flare ups during certain seasons, and for different reasons. Because of this, summer carries a high risk of danger to lupus and mixed connective tissue disorder patients in particular. Both extreme temperatures and sun exposure itself cause an already unstable body system to really go off kilter.
Because these disorders can impact the heart, kidneys and lungs, it is extemely important to try and prevent further damage to our bodies. Dehydration poses a much greater threat to us, so it’s important to remember to drink 6-8 glasses of water a day, and add other beverages such as fresh juice or refreshing iced teas.
Never go outdoors during the peak heat hours between 12 and 4 PM. Wear a big hat to shade your face, and an SPF sunscreen of at least 40. Learn to heed the signs of impending high blood pressure or kidney problems. If you begin feeling a tightness in your head, accompanied by a pounding pulse and often spots before your eyes, lie down immediately and call your physician.
If you stop urinating, or only are producing scanty amounts of urine, accompanied by intense headache, have someone drive you to the ER immediately. Do NOT try driving yourself, you may black out. As an example, I’ve been having periods of feeling as if I am going to pass out, something I’ve never had before. My husband drove me to the doctor who discovered yesterday my blood pressure is at a very dangerous 170/150. Needless to say it frightened us all out of our wits. When we asked why, she explained the combination of high heat, dehydration and constant pain from the still unhealed ankle breaks had cause my lupus to go into “crisis’ flare.
She, when asked for tips I could share with others, pointed out a few less obvious contributing factors. One is the light/heat from windows unless covered and draped. The damage from sun coming in an uncovered window is nearly twice as bad as being outoors itself. The same applies to flourescent lights. For some patients the UV light causes tremendous irritation for patients with SLE and Sjogrens.
Eyeglasses should be heavily tinted against UV rays. In Sjogren patients, who often suffer from dry aching eyes, the glasses afford them some relief and protection against the sun. Use eye moisture drops regularly to
stop the aching, stickiness and burning.
Swimming is an excellent way to ease fatigue and joint pains. During summers peak heat, try going very early in the morning, before 10 AM is perfect, or after 6 PM at night. Walking at a leisurely pace at those times is also a great way to get outdoors for a half hour or so. You might be surprised at how either of these things done two to three times a week, will improve your mood and your sense of fatigue.
Watch out for insect bites or other minor cuts or scrapes. Our systems are hyperreactive, making a small injury something to pay attention to. Especially if you are experiencing a flare up of your disease and being treated with Prednisone, Cyclosporin or other immunosuppressive treatment. Clean the area with soap and water, then lightly cover it with a bandaid treated with neosporin or other antibacterial agent.
Your appetite may be way off. So tempt yourself with small meals made up of fresh organic fruits or veggies, crackers and cheese or toasted english muffins with butter and fresh jam. If abdominal pain and diarrhea develop due to heat stressing, shower, wear something light and soft, and lie down in a darkened room. Take your meds on time, and notify your doctor if they are failing to control your pain or other symptoms.
Living with lupus or MCD doesn’t have to mean you have to avoid having summer fun. Like everything else involved with these disorders, it’s a matter of adjusting your schedule to adapt to the season, and to maintain a positive attitude. Relax, be aware of your body and don’t apologize to anyone for not being able to join in every single activity. And that means not doing things to harm yourself, just because you feel people may see you as lazy or somehow faking it. They don’t walk in your shoes, nor experience what you do. As I learned, much to my regret, is when you hurt yourself because of neglect or someones attitude towards you, you end up paying much too high a price. Enjoy life on whatever terms are safe and comfortable for you.
Fatigue is defined as the following:
[fuh-teeg] Show IPA noun, adjective, verb,-tigued, -ti·guing.
weariness from bodily or mental exertion.
a cause of weariness; slow ordeal; exertion: the fatigue ofdriving for many hours.
Physiology . temporary diminution of the irritability orfunctioning of organs, tissues, or cells after excessiveexertion or stimulation.
This is the classic definition of fatigue. For those of us who have autoimmune diseases like lupus, fatigue carries another definition. Here is my attempt to describe OUR fatigue.
Many autoimmune patients experience a form of fatigue in the course of their disease process. It is quite different from the everyday fatigue most “normal” people experience from time to time. This fatigue can engulf you and hold you hostage from daily living. Yes, I know, that is a bit dramatic but truly, it is hard to put into words how we feel.
My fatigue is different from yours. I have days where I get out of bed (Yay me!) and go to the bathroom, only to get back into bed because I cannot do anything more. Yes, this is the real fatigue. It does not mean that I slept too long. I hate hearing that. People seem to think that our fatigue is like theirs and that if we were not so lazy sleeping so much we would be better. WRONG!!!!!
If you seriously think I WANT to be in bed so much then you need to rethink your attitude. Believe me, I have mourned the loss of my abilities and I am trying to come out the other side. It can be very difficult when those around me seem to think I am making it up. I WANT to get up and participate in life!!! I WANT my old life back!!!
The fatigue I feel involves the overwhelming and complete exhaustion of my body. It is like I have run several marathons and cannot take another step. When it hits me, I literally HAVE to go to bed. No kidding. I cannot push through it. I cannot pass go or collect two hundred dollars. I HAVE to go to bed and sleep.
I have been known to sleep for a lot of hours! I can wake up and get a drink of something then back to bed I go to sleep another several hours. What happens if I push through it you say? Well, eventually, sooner than later, I will be forced into sleeping. I will fall asleep in my chair or wherever I am. My body literally will shut me down. When that happens, it takes me longer to come out on the other side. It is important for me to listen to my body and its cues so I can avoid having this happen.
In saying this I am not looking for pity. I am looking for empathy and understanding. I mean, I am not lazy! I have worked hard all my life and have had to mourn the losses of all the things I used to do. I applaud the things I can do and feel blessed for still being alive and kicking! So, if you see me out and about and happy, then know it is a good day for me and be happy! If you do not hear from me for some time, feel free to call me to check on what is happening. One phone call can make the difference in my mental status, just knowing someone cares enough to see how I am.
So, in conclusion, my fatigue is not a normal fatigue. It is all encompassing, head to toe fatigue that can put me in bed for days. Do not judge me and my fatigue. Do not belittle me or tell me to suck it up and get up. Just be supportive and care and that can make my whole day! It really is not much, don’t you think?
I must say that I have done this subject in a previous post but I found new information on a different website and thought I would share it here. It makes for interesting reading. The article cites Daniel Wallace, MD, who is one of the most widely read doctors when it comes to lupus. I hope you learn more by reading this article. It comes from www.hopkinslupus.org.
Signs, Symptoms, and Co-occuring Conditions
Lupus affects everyone differently, but certain signs and symptoms are common. [A sign is medical evidence your doctor finds during a physical exam, such as a specific rash; a symptom is a subjective indication of disease, such as joint stiffness or a headache.] In addition, other conditions, such as fibromyalgia, occur commonly in people with lupus but are not directly due to disease activity. These co-occuring conditions are known to doctors as “comorbidities.” Several signs, symptoms, and comorbidites of lupus are detailed below.
The average human body temperature is around 98.5°F, but many people run just above or below that mark. A temperature of 101°F is generally accepted as a fever. Many people with lupus experience reoccurring, low-grade temperatures that do not reach 101°. Such low-grade temperatures may signal oncoming illness or an approaching lupus flare. Fever can also signal inflammation or infection, so it is important to be aware of the patterns of your body and notify your physician of anything unusual.
Many lupus patients experience joint stiffness, especially in the morning. People often find that taking warm showers helps to relieve this problem. If this habit does not offer comfort and joint stiffness prevents you from daily activity, be sure to speak with your doctor. He/she will examine you for any signs of joint swelling and can speak with you about medications that may ease some of this pain and inflammation, such as over-the-counter pain treatments and NSAIDs. Tenderness of a joint in known as arthralgia, and it is important that your doctor distinguish this from the arthritis (true swelling) that may accompany lupus.
If you experience a fever lasting a few days or fevers that come and go over the course of a few days, you should take your temperature twice daily and keep a record. Certain trends may alert your doctor to specific processes occurring in your body. In addition, a fever of 101°F or more should be given medical attention. If you are taking steroid medications such as prednisone, be alert for any sign of infection, since steroids can suppress your immune system while also masking symptoms of infection. Immunosuppressive medications such as azathioprine, methotrexate, cyclophosphamide, and mycophenolate also suppress the immune system, so if you begin to feel ill when taking one of these medications, notify your doctor immediately.
Increased lupus activity can sometimes cause weight loss, and certain medications can cause loss of appetite. No matter what the cause of your weight loss, you should speak to your doctor to ensure that the loss does not indicate a more serious condition. If you experience a loss of appetite due to your medications, your doctor may suggest alternative medications or solutions to ease stomach discomfort.
Other medications, such as corticosteroids, can cause weight gain. Therefore, it is very important that you speak to your doctor about maintaining a balanced diet while taking these medications. You may need to reduce your calorie consumption; your physician can refer you to a nutrition counselor if needed. Light to moderate exercise can also help you to maintain a healthy weight and cardiovascular system, while also boosting your mood.
Please remember that it is very easy to gain weight, especially when taking steroids, but it is much more difficult to lose it. It is very important that you try to achieve a healthy weight, because women with lupus between the ages of 35 and 44 are fifty times more likely to experience a heart attack than the average woman. In addition, maintaining a healthy weight helps to alleviate stress on your joints and keeps your organs working productively and efficiently.
Fatigue and Malaise
Ninety percent of people with lupus will experience general fatigue and malaise at some point during the course of the disease. Some people find a short 1 ½ hour afternoon nap to be effective in reducing fatigue. However, exceeding this time frame might lead to problems sleeping at night. If you feel that you are tired throughout most of the day and that fatigue prevents you from engaging in daily activities, speak to your doctor. Fatigue accompanied by pain at certain parts of your body may be a sign of a treatable condition called fibromyalgia. Other fatigue-inducing conditions, such as anemia, low thyroid, and depression, can also be treated. If you and your doctor decide that your malaise is due solely to lupus, try to stay as active and mobile as possible during your daily routine. Often this can be difficult, but many people find that slightly pushing themselves to engage in light to moderate exercise actually increases their energy levels. However, you should never push yourself beyond reasonable discomfort.
As many as 10% of people with lupus may experience a condition called Sjogren’s syndrome, a chronic autoimmune disorder in which the glands that produce tears and saliva do not function correctly. Sjogren’s can also affect people who do not have lupus. People with Sjogren’s often experience dryness of the eyes, mouth, and vagina. They may also feel a gritty or sandy sensation in their eyes, especially in the morning. This dryness occurs because the immune system has begun to attack the moisture-producing glands of the eyes and mouth (the lacrimal and parotid glands, respectively), resulting in decreased tears and saliva.
It is important that you speak to your doctor if you experience dryness of the eyes and mouth, since the medications for these conditions must be taken on a regular basis to prevent discomfort and permanent scarring (especially of the tear glands). The Schirmer’s test is usually performed to check for Sjogren’s and involves placing a small piece of litmus paper under the eyelid. Eye symptoms can be relieved by frequent use of Artificial Tears, and an eyedrop medication called Restasis is often used to prevent worsening of Sjogren’s. Evoxac (or pilocarpine) can be used to increase both tear and saliva production, and certain lozenges (Numoisyn) can also be helpful for dry mouth.
Depression and anxiety are present in almost one third of all people with lupus. Clinical depression is different than the passing pangs of sadness that can haunt all of us from time to time. Rather, clinical depression is a prolonged, unpleasant, and disabling condition. The hallmark characteristics of depression are feelings of helplessness, hopelessness, general sadness, and a loss of interest in daily activities. Depression also often involves crying spells, changes in appetite, nonrestful sleep, loss of self-esteem, inability to concentrate, decreased interest in the outside world, memory problems, and indecision. In addition, people who are depressed may suffer from certain physiologic signs, such as headache, palpitations, loss of sexual drive, indigestion, and cramping. Patients are considered to be clinically depressed when they experience symptoms that last for several weeks and are enough to disrupt their daily lives. Patients suffering from depression also often experience a general slowing and clouding of mental functions, such as memory, concentration, and problem-solving abilities. This phenomenon is sometimes described as a “fog.” The cause of depression is not known; sometimes a genetic component predisposes an individual to the condition. Depression is almost never due to active lupus in the brain.
While clinical depression can be caused by the emotional drain of coping with a chronic medical condition and the sacrifices and adjustments that are required of the disease, it can also be induced by steroid medications (e.g., prednisone) and other physiological factors. It is important that you speak with your doctor if you feel you are experiencing clinical depression, because many people who are physically ill respond well to anti-depressant medications. In addition, your doctor may treat your depression in different ways depending on the cause.
Many people with lupus suffer from gastrointestinal problems, especially heartburn caused by gastroesophageal reflux disease (GERD). Peptic ulcers can also occur, often due to certain medications used in lupus treatment, including NSAIDs and steroids. Occasional heartburn or acid indigestion can be treated with an over-the-counter antacid, such as Rolaids, Maalox, Mylanta, or Tums. Your doctor may also include an antacid or another form of GI medication (a proton-pump inhibitor, histamine2 blocker, or promotility agent) in your treatment regimen. Antacids are effective when used to treat occasional symptoms, but you should try to avoid heartburn and acid indigestion altogether by eating smaller meals, remaining upright after eating, and cutting down on caffeine. If heartburn and acid reflux persist (e.g., for more than two weeks), you should speak with your doctor, because your heartburn symptoms could indicate a larger problem.
The thyroid is the gland in your neck associated with your metabolism—the processes by which your body makes use of energy. Autoimmune thyroid disease is common in lupus. It is believed that about 6% of people with lupus have hypothyroidism (underactive thyroid) and about 1% have hyperthyroidism (overactive thyroid). A thyroid gland that is functioning improperly can affect the function of organs such as the brain, heart, kidneys, liver, and skin. Hypothyroidism can cause weight gain, fatigue, depression, moodiness, and dry hair and skin. Hyperthyroidism can cause weight loss, heart palpitations, tremors, heat intolerance, and eventually lead to osteoporosis. Treatment for both underactive and overactive thyroid involves getting your body’s metabolism back to the normal rate. Hypothyroidism is usually treated with thyroid hormone replacement therapy. Hyperthyroidism is treated with anti-thyroid medications or radioactive iodine.
Osteoporosis (bone thinning) occurs when the bones lose calcium and other minerals that help keep them strong and compact. This condition can lead to fractures, bone pain, and shorter stature. Everyone is at risk for osteoporosis as they age, but women experience a greater risk of the condition after menopause. Studies have shown that people with lupus are at an increased risk for osteoporosis due to both the inflammation they experience with the disease and the use of prednisone.
Your bones are constantly being remodeled in a process that removes old bone cells and deposits new ones. In people with osteoporosis, the bones lose minerals faster than they can be regenerated. Medications called bisphosphonates (e.g., Actonel, Fosamax, Boniva, and Reclast) can be taken to help prevent your bones from losing calcium and other minerals by slowing or stopping the natural processes that dissolve bone tissue. In doing this, bisphosphonates help your bones remain strong and intact. If you have already developed osteoporosis, these medications may slow the thinning of your bones and help prevent bone fractures. In fact, studies have shown that bisphosphonates can lower your risk of fractured vertebrae—bone segments that make up your spine—by 50%. Similar studies demonstrate that these medications can lower the chance of breaking other bones by 30-49%. However, when bisphosphonates are not successful, patients may need a daily injection of parathyroid hormone (Forteo) to build bone.
- Wallace, Daniel J. The Lupus Book: A Guide for Patients and Their Families. 1st ed. New York: Oxford University Press, 1995.
Ok, the down time from the title of this post that probably sprang to your mind is a vacation, holiday or something along that line. How great would that be? To take a break and have some fun!
Sad to day, this down time I am talking about is not that type. When you have an autoimmune disease like lupus, one of the most frustrating symptoms is the “toxic” fatigue you can suffer at the drop of a hat. I know, fatigue is not bad, it just means you have worked hard and deserve to rest, right?
Wrong. The type of fatigue I am discussing is the all consuming, total shut down of your body where you have no control of when and how long you will be down. That is the fatigue I have had for the last five days.
It started innocently enough, just sleeping in one day. Or so I thought. It soon manifested itself into high gear and as of today, I have only been awake a total of about 20 hours in five days! No kidding! I mean, I sit in my chair for a few minutes and next thing I know, hubs is waking me up after several hours of me sleeping. I mean, I wonder when it happened and how. It is like a light switch has been flipped. I am “on” and suddenly I am “off”. It is utterly strange and scary when you have this happen.
I did call my rheumy and he said he thinks it may be both fibromyalgia and/or lupus acting up. He is hesitant to prescribe prednisone in case it is the fibromyalgia. He wants to see me on friday. Great… now another of my autoimmunes is acting up too? So in the meantime, I am taking ultram for the pain and waiting for friday. Some days I think I have forgotten how to feel “normal”.
So, as I sit here, or should I say “sleep” here, I hope things will work out and I can feel better soon. Have any of you had this toxic fatigue?
There are a lot of people out there who may wonder why I do the things I do in promoting lupus awareness. The answer is as complex at times as the disease is. In answer to those that really want to know, I will try to explain it.
I have lupus. I live with it daily. It hurts. It is unpredictable. It impacts my life every day in some way. Now, to why I seem to obsess over it, as some would think, is simple. I feel better about myself and validated by studying it and sharing with others. It gives me a sense of accomplishment and allows me to vent my anger over the things I cannot control. It helps me, plain and simple.
Another reason why I give time to this cause is because it helps me feel like a viable member of society, because it is something I know about, and can help others with. I have already had to stop working because of it but it has not stopped me from wanting to have that sense of pride in doing a good job. Self esteem it is called and I could use some since not working anymore. To feel that, I promote lupus and help others understand about it. I ask organizations for grants and donations. I call businesses and discuss participating in the walk. It helps me to feel some control over lupus and to feel that I am doing something even if I am laying in bed and in my pj’s.
You see, I was always a go-getter, the first one at work and one of the last to leave. I enjoy working. I cannot hold a “real” job anymore because of the progression of this disease and the unpredictability of it all. I mean, who would want an employee who cannot say which days they can work and for how long?
In a perfect world, I could do a job from my bed if necessary. This world, unfortunately, is not perfect, yet anyway, and so I muddle on trying to keep my brain from rotting and pushing myself to do things that bring me satisfaction.
I have decided to work, as it were, at being an advocate for lupus. If that makes me a bad person, so be it. If people think I amd welling on it too much, so sorry. I live with it every day. They don’t. If what I do is causing them to think less of me, then they do not have to read. Plain and simple, I need to feel like I am doing something about this unpredictable disease and this is how I cope. Deal with it, or not, the choice is yours.
Sorry if this sounds harsh but I have been dealing with this for a long time now and it has been bothering me. I live my life as best I can with each day and how it presents itself to me. I will not be ashamed of my feelings or my experience. Thanks for allowing me to vent.
This is one of my good friends, Angie and her story of her fight with the wolf, or lupus. Please read and learn more about lupus and the things it does to those who have it. Thanks Angie for sharing your story with us! This is her story, in her own words…
Angie’s Story of Lupus
I was diagnosed with Lupus in 1980 at the age of 8. What started as a rash on my face, which my mom thought was poison ivy, would change my life forever. I had no idea at that point what lupus was or what it could do to my body but through the years I would find out just what an ugly beast it would be…
My first flare happened at the age of 16. I developed sever swelling all over my body. At first the GP my parents took me to said that my pants were too tight (after all that was the style cause it was the 80′s). So the next time I went in I wore a dress and said “Are my pants too tight now??” I know I am a smarty pants, but I wanted the doctor to take me seriously and make the swelling go away. At that point I was placed on maxide and sent to a rheumatologist.
Let the testing begin….he checked me over and decided that I had RA. He took my blood and ran more tests and also decided that I had Mixed Connective Tissue disease. Prednisone would be my next drug that I would be placed on at that point. Prednisone made it hard to sleep and the maxide made me pee a lot. But nothing was taking the swelling away. Next I was off to a Kidney specialist. More tests…I had to give a 24 hour urine sample and more blood. He decided that there was nothing wrong with my kidneys at that point. I was then sent back to the rheumatologist and he placed me on plaquinel. The swelling went down eventually, but we never knew what had actually caused it.
Years past, I married and had two boys even though I was told I wasn’t able to have children. By the time I was 25 the swelling had returned. I went to a new GP (my other GP had passed away at this point). After testing me my GP told me that the symptoms I was having were all in my head. She gave me anti-depressants and sent me home.
This swelling and arguing with my GP went on for a year before I found out what was wrong with me. I had been working, raising my kids (they were 7 and 4 years old at the time) and having a hard time at doing those things due to the pain in my tummy. My family believed it and chalked it up to the fact that I was eating too much and exaggerating my pain. I had enough and had decided not to go back to my doctor. Then one day the pain was so intense that I made my husband take me to the hospital. The ER
nurse took one look at me and saw how jaundice I was and told me that I had to be admitted. I was in full blown liver failure and needed a transplant.
So here I am in the hospital and I am thinking…FINALLY!! Someone can help, they finally know whats wrong!!
At first I was relieved that the doctors FINALLY knew what was wrong with me and I could say to everyone “SEE?? I told you I was SICK!!” And then the fear set in…and I started thinking “OMG! I am going to die!…who will take care of my kids?? I am NOT ready to die!!” After all, I was only 25 years old. Then I hit denial, and thought this isn’t real, this isn’t happening, and after a week in the hospital I left AMA!! Maybe it was the high doses of prednisone, maybe it was that I wanted time with my family without being hooked up to IV’s? Who knows, but I knew I needed time to think and clear my head. After denial I was MAD
!! Mad at LUPUS..how dare it rear its ugly head in such a way and make me and others around me question my mortality!! I decided to fight and win!!
I was told that the closest transplant center to me was 6 hours away. I made the appointment and found that I had to wait for my insurance to clear it before I could go through the necessary check-ups and psych testing to be placed on the transplant list. After months of waiting I found myself driving to Omaha, Nebraska
for a weeks worth of what I like to call torture..the doctors call it “tests”. I was given the green light and placed on the list in November 1998.
Let the waiting and the false alarms begin…
I was given a pager/beeper (I am sure they just call ya on your cell phone now but thats how it was done then) and I had a certain amount of time to call them back. The first false alarm wasn’t too bad..they paged, I called them back and started packing. Before I could get on the road they called back to tell me that they had tested the donor liver and it wasn’t usable. So the flood of emotions that I had felt moments before (panic, excitement, fear, etc.) were reduced to tears and wonder. Would I get the call in time??
The second false alarm…
AS always the beeper went off, I called and was ready to go. But I waited. You could say I took my time getting things in order this time for fear of the let down. During the wait I called a local company of pilots that donate their time and planes to bring patients to other states. From the airport I took a cab to the hospital. I was in my gown, I had my blood work done and AGAIN the donor liver wasn’t a good one. I had to sit in the hospital and wait on someone to come get me and drive me home.
I am sure there was at least one more false alarm but I can’t recall it right now.
Or maybe I could say the third time was a charm?? Anyway, after waiting on the list for 3 years I got the call I had been waiting for. It was around midnight on February 17, 2001. My beeper went off and I thought to myself “I am tired..I don’t want to call back and get my hopes up…” I then hesitated. My mind was racing…what to do?? Should I call them? Should I even bother? Then I came to my senses and gave myself a mental slap in the face..what was I thinking?? This could be IT!! I woke my husband and called. I then realized that I didn’t have a ride. My car had broke down. So I called the first person that came to mind. My good friend Ed. He made a beeline to my house and drove me to Nebraska in record time
. Pretty sure if he had gotten pulled over he would of had a whopper of a ticket!! Laying in the backseat of his car on the way there I was praying..please God let this be it…
This was IT alright. I was prepped, taken to surgery and 14 hours later it was over. At least the surgical part was. I was told that I was within days of death and that my liver weighed over 12 pounds!! No wonder I looked pregnant!
The first few days are a blurr…and my doctors and nurses refer to them as the “honeymoon period”. Since things were going well, my husband decided to drive home and get a few more items that he needed and see our boys. He considered bringing the boys back to see me since I was doing well.
After he left my nurse decided to get me up and see if I was strong enough to walk. I guess they need to do this within 3 days of surgery due to the risk of blood clots. Anyway, the last thing I remember her saying was “Angela!! Are you ok??” Everything went black.
The weeks that followed I was in a drug induced coma. I had suffered a grand mal seizure
, collapsed lung and brain bleed (I had a stroke). I was allergic to my anti rejection medications. I had to under go plasmaferesis (procedure consists of removal of blood, separation of blood cells from plasma, and return of these blood cells to the body’s circulation, diluted with fresh plasma or a substitute.) and chemotherapy. I would have the plasmaferesis in the morning and then chemo at night. I suffered such high fevers that I had to be packed in ice. It got so bad that the hospital was preparing my family for my death.
My doctors explained to me and my family that its a miracle that I am here today. I was one in 500 million that this would even occur.(so please don’t think for an instant that this is common and will happen to you because odds are it won’t).
After 3 loooong months in ICU and the hospital I was finally able to return home. I weighed about 70 pounds (not able to eat due to chemo). I was in a wheelchair due to neuropathy caused by chemo. I was on oxygen because of my collapsed lung. And I was bald, again due to chemo.
We hadn’t told the kids that I was coming home, I wanted to surprise them. When I got there my oldest had a friend over staying the night and when he saw me he looked at Jay and said “Dude! Whats wrong with your mom??” He and my son are still friends to this day.
So that’s my story of having grown up with lupus. I hope and pray daily that I don’t suffer another flare and watch closely for the warning signs.
Today I want to share something with you all, something that means a lot to me. For quite some time now I have been posting on this blog and hoping to educate others about lupus and other autoimmune diseases. That is the reason for this blog, along with giving me an outlet to express my struggles with this disease and also my triumphs.
The reason I have the title above, is that I want to ask a question, “Have you heard about lupus?” Most will think, “yeah, I have” but can you tell me some points you may have learned from this blog? This is not a test, but look over the questions below and see how much you have learned from reading this blog. If you are unsure of an answer, the answers appear at the bottom of the post.
Thanks to everyone who reads this blog (and there are many of you out there). I hope I can make this blog more informative as I go along. Now, on to the questions…
Have you learned how long it takes to get a diagnosis of lupus (on average)?
Do you know what the life expectancy is for lupus patients?
Can you name the three types of lupus?
Do you know what part of the body lupus affects?
Is there a specific blood test for lupus?
Do you know the different drugs that can be used to treat lupus?
10 years average for a diagnosis; life expectancy is the same as normal people on average, although some will not have this result; systemic, discoid and drug induced are the three types of lupus; lupus can affect almost any part of the body; no, there is no one test that can tell you if you have lupus, but an overview of symptomology and tests can prove the diagnosis; there are many and range from over the counter nsaids to steroids and chemotherapy agents.
I have found a way to feel better, or at least a little better than normal these days. My normal for the past few months has been a flare with no end in sight. Painful and depressing. For the last few days though, I decided to try a new trick against the wolf. What is it?
Each and every day for the last few days I have made myself lay down for an hour or so and take a nap. Yup, a nap. Guess what happened? Well, I have always felt at my best right after waking, before the pain gets going full tilt. By taking a midday nap, it makes my body feel as it does in the mornings, and I get decently lower pain. Will this keep up? I do not know. For now though, I will take the naps and see if wolfie will lay off of me for a bit.
I wanted to share this because it might help someone else out there too. I figure, like most of us with lupus, that it is worth a shot to try it. Believe me, we try all kinds of things in an attempt to get this disease under control. This is an easy one to try though. Happy napping!
Behcets Disease is another of the autoimmune diseases family. Please read and become more informed. This information is from the website www.behcets.com.
Behçet’s disease is common in the Middle East, Asia, and Japan. It is rare in the United States. In Middle Eastern and Asian countries, the disease affects more men than women. In the United States, it affects more women than men. Behçet’s disease tends to develop in people in their 20′s or 30′s, but people of all ages can develop this disease. Behçet’s disease is an autoimmune disease that results from damage to blood vessels throughout the body, particularly veins. In an autoimmune disease, the immune system attacks and harms the bodies’ own tissues. The exact cause of Behçet’s disease is unknown. Most symptoms of the disease are caused by vasculitis (an inflammation of the blood vessels). Inflammation is a characteristic reaction of the body to injury or disease and is marked by four signs: swelling, redness, heat, and pain. Doctors think that an autoimmune reaction may cause the blood vessels to become inflamed, but they do not know what triggers this reaction. Under normal conditions, the immune system protects the body from diseases and infections by killing harmful “foreign” substances, such as germs, that enter the body. In an autoimmune reaction, the immune system mistakenly attacks and harms the body’s own tissues. Behçet’s disease is not contagious; it is not spread from one person to another. Behçet’s disease affects each person differently. The four most common symptoms (as listed) are mouth sores, genital sores, inflammation inside of the eye, and skin problems. Inflammation inside of the eye (uveitis, retinitis, and iritis) occurs in more that half of those with Behçet’s disease and can cause blurred vision, pain, and redness. Other symptoms may include arthritis, blood clots, and inflammation in the central nervous system and digestive organs.
Behcet’s disease has the ability to involve blood vessels of nearly all sizes and types, ranging from small arteries to large ones, and involving veins as well as arteries. Because of the diversity of blood vessels it affects, manifestations of Behcet’s may occur at many sites throughout the body. However, the disease does seem to target certain organs and tissues; these are described below:
Behcet’s may cause either anterior uveitis (inflammation in the front of the eye) or posterior uveitis (inflammation in the back of the eye), and sometimes causes both at the same time.
Anterior uveitis results in pain, blurry vision, light sensitivity, tearing, or redness of the eye.
Posterior uveitis may be more dangerous and vision–threatening because it often causes fewer symptoms while damaging a crucial part of the eye — the retina.
Painful sores in the mouth called “aphthous ulcers” (known as oral aphthosis [af-THO-sis] and aphthous stomatitis) affect almost all patients with Behçet’s disease. Individual sores or ulcers are usually identical to canker sores, which are common in many people. These sores are usually a result of minor trauma. They are often the first symptom that a person notices and may occur long before any other symptoms appear. However, the lesions are more numerous, more frequent, and often larger and more painful. Aphthous ulcers can be found on the lips, tongue, and inside of the cheek. Aphthous ulcers may occur singly or in clusters, but occur in virtually all patients with Behcet’s. The sores usually have a red border and several may appear at the same time. They may be painful and can make eating difficult. Mouth sores go away in 10 to 14 days but often come back. Small sores usually heal without scarring, but larger sores may scar.
Skin problems are a common symptom of Behçet’s disease. Skin sores often look red or resemble pus-filled bumps or a bruise. The sores are red and raised, and typically appear on the legs and on the upper torso. In some people, sores or lesions may appear when the skin is scratched or pricked. When doctors suspect that a person has Behçet’s disease, they may perform a pathergy test, in which they prick the skin with a small needle; 1 to 2 days after the test, people with Behçet’s disease may develop a red bump where the doctor pricked the skin. However, only half of the Behçet’s patients in Middle Eastern countries and Japan have this reaction. It is less commonly observed in patients from the United States, but if this reaction occurs, then Behçet’s disease is likely.
Pustular skin lesions that resemble acne, but can occur nearly anywhere on the body. This rash is sometimes called “folliculitis”.
Skin lesions called erythema nodosum: red, tender nodules that usually occur on the legs and ankles but also appear sometimes on the face, neck, or arms. Unlike erythema nodosum associated with other diseases (which heal without scars), the lesions of Behcet’s disease frequently ulcerate.
Arthritis or “arthralgias” which is inflammation of the joints, occurs in more than half of all patients with Behçet’s disease. Arthritis causes pain, swelling, and stiffness in the joints, especially in the knees, ankles, wrists, and elbows. Arthritis that results from Behçet’s disease usually lasts a few weeks and does not cause permanent damage to the joints.
Behçet’s disease affects the central nervous system in about 23 percent of all patients with the disease in the United States. The central nervous system includes the brain and spinal cord. Its function is to process information and coordinate thinking, behavior, sensation, and movement. Behçet’s disease can cause inflammation of the brain and the thin membrane that covers and protects the brain and spinal cord. This condition is called meningoencephalitis. People with meningoencephalitis may have fever, headache, stiff neck, and difficulty coordinating movement, and should report any of these symptoms to their doctor immediately. If this condition is left untreated, a stroke (blockage or rupture of blood vessels in the brain) can result.
Central nervous system involvement is one of the most dangerous manifestations of Behcet’s. The disease tends to involve the “white matter” portion of the brain and brainstem, and may lead to headaches, confusion, strokes, personality changes, and (rarely) dementia. Behcet’s may also involve the protective layers around the brain (the meninges), leading to meningitis. Because the meningitis of Behcet’s disease is not associated with any known infection, it is often referred to as “aseptic” meningitis.
Genital sores affect more than half of all people with Behçet’s disease. The sores look similar to the mouth sores and may be painful. After several outbreaks, they may cause scarring.
Male — painful genital lesions that form on the scrotum, similar to oral lesions, but deeper.
Female — painful genital ulcers that develop on the vulva.
Behçet’s disease causes inflammation and ulceration (sores) throughout the digestive tract that are identical to the aphthous lesions in the mouth and genital area.
Ulcerations may occur anywhere in the gastrointestinal tract from the mouth to the anus. This leads to abdominal pain, diarrhea, and/or bleeding. Because these symptoms are very similar to symptoms of other diseases of the digestive tract, such as ulcerative colitis and Crohn’s disease, careful evaluation is essential to rule out these other diseases.
Above is a colonoscopy of a Behcet’s Disease Patient – showing ulcers.
Diagnosing Behçet’s disease is very difficult because no specific test confirms it. When a patient reports symptoms, the doctor must examine the patient and rule out other conditions with similar symptoms. Because it may take several months or even years for all the common symptoms to appear, the diagnosis may not be made for a long time and is often a retrospective diagnosis. A patient may even visit several different kinds of doctors before the diagnosis is made.
Criteria for Behçet’s disease:
- Mouth sores (oral ulcers) at least three times in 12 months
- Any two of the following:
- Recurring genital sores/ulcers
- Eye inflammation with loss of vision
- Characteristic skin lesions
- Positive pathergy (skin prick test)
Besides finding these signs, the doctor must rule out other conditions with similar symptoms, such as Lupus, Crohn’s disease, and Rheumatoid Arthritis. The doctor also may recommend that the patient see an ophthalmologist to identify possible complications related to eye inflammation. A dermatologist may perform a biopsy of mouth, genital, or skin lesions to help distinguish Behçet’s from other disorders.
There is no specific “Behçet’s’test”. Consequently, the diagnosis is based on the occurrence of symptoms and signs that are compatible with the disease, the presence of certain features that are particularly characteristic (e.g., oral or genital ulcerations), elimination of other possible causes of the patient’s presentation, and – whenever possible – proof of vasculitis by biopsy of an involved organ.
International Study Group for Behçet’s Disease
An international group of physicians has established a set of guidelines to aid in the classification of Behçet’s patients. This study group created the criteria for the purpose of conducting research on the disease. The criteria put forth by the study group include:
In addition, a patient must also meet two of the following:
recurrent genital ulcerations
eye lesions (uveitis or retinal vasculitis) observed by an opthalmologist
skin lesions (erythema nodosum, pseudofolliculitis, papulopustular lesions, acneiform nodules) adult patients not on corticosteroids
positive “pathergy test” read by a physician within 24-48 hours of testing
* It is important to note that the criteria set forth by the International Study Group of Physicians was intended for classification of patients for research only, not for a Behçet’s diagnosis.
The pathergy test is a simple test in which the forearm is pricked with a small, sterile needle. Occurrence of a small red bump or pustule at the site of needle insertion, 1 to 2 days after the test, constitutes a positive test. Although a positive pathergy test is helpful in the diagnosis of Behçet’s disease, only a minority of Behçet’s patients demonstrate the pathergy phenomenon (i.e., have positive tests). Patients from the Mediterranean region are more likely to demonstrate a positive response to a pathergy test. However, only 50% of patients in Middle Eastern countries and Japan have this reaction. This reaction is even less common in the United States. In addition, other conditions can occasionally result in positive pathergy tests, so the test is not 100% specific.
Pictured below is an example of the pathergy test: 1) taken at the time when the patient was “stuck” with the sterile needle; 2) the area immediately after the stick; 3) & 4) show the area one day and two days after the needle stick, respectively.
After the Diagnosis
Most people with Behçet’s disease can lead productive lives and control symptoms with proper medication, rest, and exercise. There are many medicines available to doctors to use to relieve pain, treat symptoms, and prevent complications. When treatment is effective, flares usually become less frequent. Many patients eventually enter a period of remission (a disappearance of symptoms). Sometimes, treatment does not relieve symptoms, and gradually more serious symptoms such as eye disease may occur. Serious symptoms may appear months or years after the first signs of Behçet’s disease occur.
Researchers are exploring possible genetic, bacterial, and viral causes of Behçet’s disease as well as improved drug treatment. Researchers are also investigating factors in the environment, such as bacterial or viral factors, that may trigger Behçet’s disease. In addition, researchers are identifying other medicines to better treat Behçet’s disease.
TREATMENTS FOR BEHÇET’S DISEASE
There is no cure for Behçet’s disease. Treatment typically focuses on reducing discomfort and preventing serious complications. Corticosteroids and other medications that suppress the immune system may be prescribed to treat inflammation. Behçet’s is a chronic disease that recurs. However, patients may have periods of time when symptoms go away temporarily (remission). The severity of the disease varies from patient to patient. Some patients may live somewhat normal lives, but others may become blind or severely disabled.
Behçet’s disease affects different parts of the body, therefore, a patient probably will see several different doctors. It may be helpful to both the doctors and the patient for one doctor to manage the complete treatment plan. This doctor can coordinate the treatments and monitor any side effects from the various medications that the patient takes.
A rheumatologist (a doctor specializing in arthritis and other inflammatory disorders) often manages a patient’s treatment and treats joint disease. The following specialists also treat other symptoms that affect the different body systems:
- Gynecologist-treats genital sores in women
- Urologist-treats genital sores in men
- Dermatologist-treats genital sores in men and women, and skin and mucous membrane problems
- Ophthalmologist-treats eye inflammation
- Gastroenterologist-treats digestive tract symptoms
- Hematologist-treats disorders of the blood
- Neurologist-treats central nervous system symptoms
Although there is no cure for Behçet’s disease, people usually can control symptoms with proper medication, rest, exercise, and a healthy lifestyle. The goal of treatment is to reduce discomfort and prevent serious complications such as disability from arthritis or blindness. The type of medicine and the length of treatment depend on the person’s symptoms and their severity.It is likely that a combination of treatments will be needed to relieve specific symptoms. Patients should tell each of their doctors about all of the medicines they are taking so that the doctors can coordinate treatment.
Topical medicine is applied directly on the sores to relieve pain and discomfort. For example, doctors prescribe rinses, gels, or ointments. Creams are used to treat skin and genital sores. The medicine usually contains corticosteroids (which reduce inflammation), other anti-inflammatory drugs, or an anesthetic, which relieves pain.
Doctors also prescribe medicines taken by mouth to reduce inflammation throughout the body, suppress the overactive immune system, and relieve symptoms. Doctors may prescribe one or more of the medicines described below to treat the various symptoms of Behçet’s disease. The treatment of Behçet’s syndrome depends on the severity and the location of its manifestations in an individual patient.
Steroid (cortisone) gels, pastes (such as Kenalog in Orabase) and creams can be helpful for the mouth and genital ulcers. Colchicine can also minimize recurrent ulcerations. Mouth and genital ulcers healed and were reported at a national meeting of the American College of Rheumatology as less frequent in 9 or 12 patients who were treated with Trental (pentoxifylline). Trental also seemed to maintain the healed ulcers for up to the 29 months of the study. The effectiveness of Trental, the researchers said, seemed to be enhanced by the combination with colchicine in some patients.
Joint inflammation can require non-steroidal anti-inflammatory drugs (such as ibuprofen and others) or oral steroids. Colchicine and oral and injectable cortisone are used for inflammation involving the joints, eyes, skin, and brain. Sulfasalazine has been effective in some patients for arthritis.
- Corticosteroids – Prednisone is a corticosteroid prescribed to reduce pain and inflammation throughout the body for people with severe joint pain, skin sores, eye disease, or central nervous system symptoms. Patients must carefully follow the doctor’s instructions about when to take prednisone and how much to take. It also is important not to stop taking the medicine suddenly, because the medicine alters the body’s production of the natural corticosteroid hormones. Long-term use of prednisone can have side effects such as osteoporosis (a disease that leads to bone fragility), weight gain, delayed wound healing, persistent heartburn, and elevated blood pressure. However, these side effects are rare when prednisone is taken at low doses for a short time. It is important that patients see their doctor regularly to monitor possible side effects. Corticosteroids are useful in early stages of disease and for acute severe flares. They are of limited use for long-term management of central nervous system and serious eye complications.
- Immunosuppressive drugs - These medicines (in addition to corticosteriods) help control an overactive immune system, which occurs in Behçet’s disease, and reduce inflammation throughout the body, and can lessen the number of disease flares. Doctors may use immunosuppressive drugs when a person has eye disease or central nervous system involvement. These medicines are very strong and can have serious side effects. Patients must see their doctor regularly for blood tests to detect and monitor side effects.
Doctors may use one or more of the following drugs depending on the person’s specific symptoms.
- Azathioprine (Imuran) - classified as an immunosuppressant medication. Azathioprine is used to suppress the immune system in patients who have had kidney transplants. Although its exact mechanism of action in rheumatoid arthritis is not known, its effect in suppressing the immune system appears to decrease the activity of this illness. Most commonly prescribed for people with organ transplants because it suppresses the immune system, azathioprine is now used for people with Behçet’s disease to treat uveitis and other uncontrolled disease manifestations. This medicine can upset the stomach and may reduce production of new blood cells by the bone marrow.
- Chlorambucil - Doctors may use these drugs to treat uveitis and meningoencephalitis. People taking either agent must see their doctor frequently because either can have serious side effects, such as permanent sterility and cancers of the blood. Patients have regular blood tests to monitor blood counts of white cells and platelets.
- Colchicine - Commonly used to treat gout, which is a form of arthritis, colchicine reduces inflammation throughout the body. The medicine sometimes is used to treat arthritis, mucous membrane, and skin symptoms in patients with Behçet’s disease. A research study in Turkey suggested that the medication works best for males with the disorder. Common side effects of colchicine include nausea, vomiting, and diarrhea. The doctor can decrease the dose to relieve these side effects.
- Cyclophosphamide - drug that is used primarily for treating several types of cancer. In order to work, cyclophosphamide first is converted by the liver into two chemicals, acrolein and phosphoramide. Acrolein and phosphoramide are the active compounds, and they slow the growth of cancer cells by interfering with the actions of deoxyribonucleic acid (DNA) within the cancerous cells. It is, therefore, referred to as a cytotoxic drug. Unfortunately, normal cells also are affected, and this results in serious side effects. Cytoxan also suppresses the immune system and is also referred to as immunosuppressive.
- Cyclosporine - Like azathioprine, doctors prescribe this medicine for people with organ transplants. When used by patients with Behçet’s disease, cyclosporine reduces uveitis and uncontrolled disease in other organs. To reduce the risk of side effects, such as kidney and liver disease, the doctor can adjust the dose. Patients must tell their doctor if they take any other medicines, because some medicines affect the way the body uses cyclosporine.
- Enbrel (Etanetcept) - Etanercept is an injectable drug that blocks tumor necrosis factor alpha (TNF alpha) and is used for treating rheumatoid arthritis, ankylosing spondylitis, and psoriatic arthritis. TNF alpha is a protein that the body produces during the inflammatory response, the body’s reaction to injury. TNF alpha promotes the inflammation and its associated fever and signs (pain, tenderness, and swelling) in several inflammatory conditions including rheumatoid arthritis and ankylosing spondylitis. Etanercept is a synthetic (man-made) protein that binds to TNF alpha. It thereby acts like a sponge to remove most of the TNF alpha molecules from the joints and blood. This prevents TNF alpha from promoting inflammation and the fever, pain, tenderness and swelling of joints in patients with rheumatoid or psoriatic arthritis and ankylosing spondylitis. Etanercept reduces the signs and symptoms of rheumatoid arthritis, the arthritis of psoriasis, and ankylosing spondylitis. It prevents the progressive destruction of the joints in patients with rheumatoid arthritis and the arthritis of psoriasis.
- Interferon - are multiple substances naturally produced by cells in the body to help fight infections and tumors. They may also be synthetic (man-made) versions of these substances. Alpha interferons, such as Roferon-A, Intron-A, and Alferon-N, are used to treat hairy cell leukemia, malignant melanoma, and AIDS-related Kaposi’s sarcoma. They are also used to treat laryngeal papillomatosis (growths in the respiratory tract) in children, genital warts, and some kinds of hepatitis. Gamma interferon, like Actimmune, is a synthetic (man-made) version of a substance naturally produced by cells in the body to help fight infections and tumors. Gamma interferon is used to treat chronic granulomatous disease and osteopetrosis. Interferon beta-1a, like Avonex and Rebif, is used to treat the relapsing forms of multiple sclerosis (MS) and genital warts. This medicine will not cure MS, but it may slow some disabling effects and decrease the number of relapses of the disease. Interferon beta-1b, such as Betaseron, is also used to treat the relapsing-remitting form of multiple sclerosis (MS). Again, this medicine will not cure MS, but may decrease the number of relapses of the disease. There are no generic forms of Interferon available.
- Kenalog (Triamcinolone) – a topical steroid. It reduces or inhibits the actions of chemicals in the body that cause inflammation, redness, and swelling. It is used to treat the inflammation caused by a number of conditions such as allergic reactions, eczema, and psoriasis.
- Methotrexate - Methotrexate is classified as an antimetabolite drug, which means it is capable of blocking the metabolism of cells. It has been found very helpful in treating rheumatoid arthritis. It seems to work, in part by altering aspects of immune function which may play a role in causing rheumatoid arthritis.
- Prednisone - is an oral, synthetic (man-made) corticosteroid used for suppressing the immune system and inflammation. It has effects similar to other corticosteroids such as triamcinolone (Kenacort), methylprednisolone (Medrol), prednisolone (Prelone) and dexamethasone (Decadron). These synthetic corticosteroids mimic the action of cortisol (hydrocortisone), the naturally-occurring corticosteroid produced in the body by the adrenal glands. Corticosteroids have many effects on the body, but they most often are used for their potent anti-inflammatory effects, particularly in those conditions in which the immune system plays an important role. Such conditions include arthritis, colitis, asthma, bronchitis, certain skin rashes, and allergic or inflammatory conditions of the nose and eyes. Prednisone is inactive in the body and, in order to be effective, first must be converted to prednisolone by enzymes in the liver. Therefore, prednisone may not work as effectively in people with liver disease whose ability to convert prednisone to prednisolone is impaired.
- Remicade (Infliximab) - Infliximab is an injectable antibody that blocks the effects of tumor necrosis factor alpha (TNF-alpha). By blocking the action of TNF-alpha, infliximab reduces the signs and symptoms of inflammation. This medication is administered via an IV-infusion.
- Sulfasalazine – a prodrug, that is, it is not active in its ingested form. It is broken down by bacteria in the colon into two products: 5-aminosalicylic acid (5ASA), and sulfapyridine. There is some controversy as to which of these two products are responsible for the activity of azulfidine. Whereas it is known that 5ASA has therapeutic benefit, it is not clear whether sulfapyridine adds any further benefit. In the colon, the products created by the breakdown of sulfasalazine work as anti-inflammatory agents for treating inflammation of the colon. The beneficial effect of sulfasalazine is believed to be due to a local effect on the bowel, although there may also be a beneficial systemic immune-suppressant effect as well. Following oral administration, 33% of the sulfasalazine is absorbed, all of the sulfapyridine is absorbed, and about 33% of the 5ASA is absorbed. Sulfasalazine was approved by the FDA in 1950.
- CellCept – contains the active ingredient mycophenolate mofetil. Cellcept belongs to a group of medicines called immunosuppressants. Immunosuppressants are used to prevent rejection of transplanted organs, and work by stopping your immune system from reacting to the transplanted organ. Cellcept may be used together with other medicines known as cyclosporin and corticosteroids.
- Thalidomide – Thalidomide is an oral medication used for treating the skin conditions of leprosy, a disease caused by a parasite, Mycobacterium leprae. The mechanism of action of thalidomide is not well understood. The immune system reaction to Mycobacterium leprae plays an important role in producing the skin manifestations of leprosy. Scientists believe that thalidomide modifies the reaction of the immune system to Mycobacterium leprae and thereby suppresses the skin reaction. Thalidomide also is being evaluated as a treatment for HIV. Thalidomide was approved by the FDA in July, 1998.
- Trental (Pentoxifylline) - decreases the “stickiness” (viscosity) of blood and thereby improves its flow. This increase blood flow helps patients with peripheral arterial disease to obtain better circulation and oxygen delivery to vital tissues. Pentoxifylline is used in patients to treat a condition of painful legs that develop with exercise because of inadequate circulation to the legs and feet.
- Combination Treatment – Cyclosporine is sometimes used together with azathioprine when one medication fails to work by itself. A common combination is prednisone along with an immunosuppressive drug.
Rest and Exercise
Although rest is important during flares, doctors usually recommend moderate exercise, such as swimming or walking, when the symptoms have improved or disappeared. Exercise can help people with Behçet’s disease keep their joints strong and flexible.
This time I looked up celiac disease since it is another of the autoimmune diseases and can overlap with lupus. I would also like to state that my baby sister has this and it is found in 1 out of every 133 people. It does not get a lot of attention, like lupus, yet it affects so many people. In an effort to raise awareness of autoimmune diseases other than lupus, I have found this information to read. It is amazing that there are so many autoimmune diseases out there! This information was obtained from the website, www.celiac.org. If you would like more information, please see their site. Thanks and good reading!
Celiac Disease (CD) is a lifelong inherited autoimmune condition affecting children and adults. When people with CD eat foods that contain gluten, it creates an immune-mediated toxic reaction that causes damage to the small intestine and does not allow food to be properly absorbed. Even small amounts of gluten in foods can affect those with CD and cause health problems. Damage can occur to the small bowel even when there are no symptoms present.
Gluten is the common name for the proteins in specific grains that are harmful to persons with celiac disease. These proteins are found in ALL forms of wheat (including durum, semolina, spelt, kamut, einkorn and faro) and related grains rye, barley and triticale and MUST be eliminated.
The cause of Celiac Disease (CD), also known as celiac sprue or gluten sensitive enteropathy (GSE), is still a mystery. One out of 133 people in the United States is affected with celiac disease. CD occurs in 5-15% of the offspring and siblings of a person with celiac disease. In 70% of identical twin pairs, both twins have the disease. It is strongly suggested that family members be tested, even if asymptomatic. Family members who have an autoimmune disease are at a 25% increased risk of having celiac disease.
Celiac Disease is not a food allergy – it is an autoimmune disease. Food allergies, including wheat allergy, are conditions that people can sometimes grow out of. This is not the case with Celiac Disease.
Celiac Disease (CD) is unique in that a specific food component, gluten, has been identified as the trigger. When individuals with CD eat gluten, the villi (tiny hair-like projections in the small intestine that absorb nutrients from food) are damaged. This is due to an autoimmune reaction to gluten. Damaged villi do not effectively absorb basic nutrients – proteins, carbohydrates, fats, vitamins, minerals and, in some cases, water and bile salts. If CD is left untreated, damage to the small bowel can be chronic and life threatening, causing an increased risk of associated disorders – both nutritional and immune related.
Dermatitis Herpetiformis (DH) is the skin manifestation of celiac disease characterized by blistering, intensely itchy skin. The rash has a symmetrical distribution and is most frequently found on the face, elbows, knees and buttocks. DH patients can have intestinal damage without obvious gastrointestinal symptoms.
Dermatitis Herpetiformis (DH) is diagnosed by a biopsy of a skin lesion and staining for IgA in the tissues. More than 85% of DH patients have small bowel sensitivity to gluten. Everyone with DH needs to follow a gluten-free diet.
ASSOCIATED AUTOIMMUNE DISORDERS
Insulin-dependent Type 1 Diabetes Mellitus, Liver diseases, Thyroid Disease-Hashimoto’s Thyroiditis, Lupus (SLE), Addison’s Disease, Chronic Active Hepatitis, Rheumatoid Arthritis, Turner Syndrome, Sjögren’s Syndrome, Raynaud’s Syndrome, Alopecia Areata and Scleroderma
OTHER DISORDERS LINKED WITH CELIAC DISEASE
Down Syndrome, Fibromyalgia, Chronic Fatigue Syndrome, Williams Syndrome
Celiac Disease can appear at any time in a person’s life. In adults, the disease can be triggered for the first time after surgery, viral infection, severe emotional stress, pregnancy or childbirth. CD is a multi-system, multi-symptom disorder. Symptoms vary and are not always gastrointestinal (GI). GI symptoms can often mimic other bowel disorders.
Infants, toddlers and young children with CD may often exhibit growth failure, vomiting, bloated abdomen, behavioral changes and failure to thrive.
CLASSIC SYMPTOMS MAY INCLUDE
- Abdominal cramping, intestinal gas
- Distention and bloating of the stomach
- Chronic diarrhea or constipation (or both)
- Steatorrhea – fatty stools
- Anemia – unexplained, due to folic acid, B12 or iron deficiency (or all)
- Unexplained weight loss with large appetite or weight gain
- Dental enamel defects
- Osteopenia, osteoporosis
- Bone or joint pain
- Fatigue, weakness and lack of energy
- Infertility – male/female
- Mouth ulcers
- Delayed puberty
- Tingling or numbness in hands or feet
- Migraine headaches
SOME LONG-TERM CONDITIONS THAT CAN RESULT FROM UNTREATED CD
- Iron deficiency anemia
- Early onset osteoporosis or osteopenia
- Vitamin K deficiency associated with risk for hemorrhaging
- Vitamin and mineral deficiencies
- Central and peripheral nervous system disorders – usually due to unsuspected nutrient deficiencies
- Pancreatic insufficiency
- Intestinal lymphomas and other GI cancers (malignancies)
- Gall bladder malfunction
- Neurological manifestations
A person seeking diagnosis MUST be following a daily diet that contains gluten for at least 4 weeks in order for test results to be accurate. Specific antibody blood tests are the initial step in screening for CD. Patients should always consult with a physician to ensure proper diagnosis.
Recommended Blood Tests:
- Anti-tissue transglutaminase antibody (tTG – IgA and IgG)
commonly used whether or not symptoms are present and the most sensitive test available
- Anti-endomysial antibody (EMA-IgA) – highly specific marker for celiac disease
- Anti-deaminated gliadin peptide (DGP – IgA and IgG)
used when tTG or EMA is negative and in cases where patient is IgA deficient
- Total serum IgA – used to check levels to exclude selective IgA deficiency that results in a false negative test
- Anti-gliadin antibody (AgA – IgG and IgA) not considered sensitive or specific enough for adults, but used for children under 2 because tTG and EMA antibodies may be absent. The anti-DGP test is sensitive in this group.
A patient with positive antibody tests and a patient with selective IgA deficiency are strongly advised to consult with their physician regarding a small bowel biopsy (which is performed endoscopically). A positive small bowel biopsy is required to confirm the diagnosis and assess the degree of damage to the villi in the intestinal lining. Antibody test results can only suggest the presence of Celiac Disease but cannot confirm it. When antibody results and biopsy are inconclusive, or when the patient is on a gluten-free diet, genetic testing of the HLA (human leukocyte antigen) DQ2/DQ8 genes may be helpful. The specific genes DQ2 and/or DQ8 are considered necessary for Celiac Disease to develop. Since one-third of the population also has these genes, the presence of DQ2 or DQ8 does not imply that the person will necessarily develop CD, rather, that they have a genetic predisposition to CD.
Genetic testing does not diagnose Celiac Disease – its largest benefit is that the absence of DQ2 and DQ8 essentially excludes CD.
The onset of Celiac Disease can occur at any time in a person’s life. Once a person is diagnosed, family members should be urged to get tested as well.
Celiac Disease/Dermatitis Herpetiformis (CD/DH) are chronic disorders. The only treatment is the lifelong adherence to the gluten-free diet. When gluten is removed from the diet, the small intestine will start to heal and overall health improves. Medication is not normally required. Consult your physician regarding specific nutritional supplementation to correct any deficiencies. The diagnosed celiac should have medical follow-up to monitor the clinical response to the gluten-free diet.
Adapting to the gluten-free diet requires some lifestyle changes. It is essential to read labels which are often imprecise, and to learn how to identify ingredients that may contain hidden gluten. Even small amounts of ingested gluten can affect those with CD and cause health problems.
Dietary compliance increases the quality of life and decreases the likelihood of osteoporosis, intestinal lymphoma and other associated illnesses.
Because osteoporosis is common and may be profound in patients with newly diagnosed CD, bone density should be measured at or shortly after diagnosis.
Potential harmful ingredients include:
- unidentified starch
Gluten may also be used as a binder in some pharmaceutical products. Request clarification from food and drug manufacturers when necessary.
Once again, I found a great article that discusses depression in lupus patients. This information is from the website:
Lupus Disease Activity May Cause, Worsen Depression
Both negative life events and lupus disease activity may be capable of contributing to major depression in individuals with lupus.
Fabiano Nery, M.D., a Brazilian psychiatrist who also completed a residency in internal medicine, has long been fascinated by the interface between psychiatric and medical illnesses—and especially by the interface between depression and the autoimmune disease systemic lupus erythematosus.
In 2004, while affiliated with the University of Sao Paulo Medical School, he designed a study to get more insight into possible causes of depression in lupus patients—say, stressful life events or damage to the brain inflicted by lupus. He was particularly interested in the latter possibility since lupus, unlike the autoimmune disease rheumatoid arthritis, is known to be capable of damaging the brain. The brain involvement can be either widespread or focal and may involve inflammation of small blood vessels or interaction of autoantibodies with antigens on neuronal cell membranes.
Seventy-one subjects with lupus were evaluated for the presence of major depressive disorder, the intensity of depression, life events during the previous six months that had had a negative and pronounced impact on their lives, and lupus disease activity. Other health information about the subjects, such as medication use, was also collected.
Sixteen of the 71 subjects (23 percent) met DSM-IV diagnostic criteria for a current major depressive disorder. This finding did not surprise the investigators since other studies have found high rates of major depression in lupus subjects.
Prednisone, a medication used to treat lupus and known sometimes to produce depression, did not seem to explain this high rate of depression because the amount of prednisone used by subjects with and without depression was essentially the same. Yet nine of the 16 subjects with major depression reported having experienced at least one major negative life event during the previous six months, compared with 13 of the 55 subjects without a major depression—a statistically significant difference. This finding suggested that potent negative events might have precipitated the depression. Also, the severity of subjects’ depression was associated with having experienced negative life events, which likewise strengthened the argument that negative events could trigger such depression.
Subjects with major depression, however, had more severe lupus disease activity than did those without a major depression, with a trend toward statistical significance. This finding implied that lupus disease activity might have triggered the depression, and another result bolstered this possibility: Even when stressful life events were taken into consideration, there was still a highly significant link between depression severity and disease activity.
Thus, it looks as if both negative life events and lupus disease activity may lead to major depression in lupus patients, Nery and his group concluded in their study, which is in press with Comprehensive Psychiatry.
Nonetheless, as Nery told Psychiatric News, “if a lupus flare-up can truly trigger depression, then there is still the question of whether the depression is due to brain damage by the disease or whether the patient becomes depressed in reaction to the disease. I would say that both can occur, probably at the same time in the same patient.
“My plan,” he continued, “is to use neuroimaging tools to determine whether specific brain areas associated with mood regulation are damaged during a lupus flare, and try to use these tools to disentangle factors such as psychosocial stress and biological and psychological factors in the development of mood disorders.”
When asked whether these findings have any practical implications for clinical psychiatrists, he replied, “Yes, absolutely. When evaluating depressed patients with systemic lupus erythematosus, consider that the disease activity is an important risk factor for the worsening of the depression.”
The study was funded by Fundacao de Amparo a Pesquisa do Estado de Sao Paulo and Conselho de Desenvolvimento Tecnologico e Cientifico.
An abstract of “Major Depressive Disorder and Disease Activity in Systemic Lupus Erythematosus” can be accessed atwww.sciencedirect.com> by clicking on “Browse A-Z of journals,” then “C,” then “Comprehensive Psychiatry.” ▪
Once again, in researching some things about lupus, I found this interesting article discussing neurology and lupus. It is written by neurologists for neurologists but it is enlightening for patients as well. This information came fromt he website www.asktheneurologist.com.
“Lupus neurology:- Here we deal with all the neurological manifestations of lupus including epilepsy, headache,and psychiatric problems“
Systemic Lupus Erythematosus (SLE or lupus for short) is disease of the immune system (autoimmune disease) affecting many different organ systems throughout the body.
Neurological and psychiatric symptoms occur in many patients due to the disease process itself however the issue is further complicated by the fact that drugs used in SLE and other “rheumatological” conditions may have a variety of neurological side effects. In addition neurological problems may result from damage to other organ systems such as the liver and kidneys.
Lupus Neurology:- Neurological features of SLE
SLE is characterized by the presence of circulating antibodies which attack the host (“autoantibodies”)
A wide variety of neurological symptoms may occur in patients previously diagnosed with SLE. However, patients ultimately diagnosed with SLE may initially present with neurological symptoms and have no obvious involvement of other organ systems on initial clinical evaluation. Therefore many patients originally going to a neurologist with neurological syndromes should be screened for clinical and laboratory features of SLE.
On the other hand, neurological evaluation of patients with established SLE may not be straightforward, as neurological problems may be a direct consequence of the disease, a result of other organ involvement or due to therapeutic interventions. The American College of Rheumatology defined 19 neuropsychiatric syndromes (NPS) occurring in SLE. These represent conditions directly associated with SLE and does not include conditions occurring because of other organ involvement or therapy.
The antiphospholipid antibody syndrome (APLAS) is a frequent accompaniment to SLE and the frequency of nervous system involvement is higher in SLE patients with antiphospholipid antibodies, particularly anticardiolipin antibodies. Furthermore, neuropsychiatric syndromes frequently occur in APLAS without SLE
Various neurological problems may be interrelated. For example, cerebrovascular disease or stroke may lead to “focal deficits” such as weakness of one side, cognitive problems, seizures and a movement disorder . Similarly, inflammation of the spinal cord (“myelitis”) may either be isolated, or reflect the co-existence of Neuromyelitis optica or Devic disease, or multiple sclerosis (MS) both of which which are sometimes an issue in “lupus neurology”.
A severe syndrome of short term memory loss due to bilateral hippocampal inflammation known as “limbic encephalitis” may occasionally occur.
Although headache is often considered to be one of the neurological manifestations of SLE the association is controversial. Headache is probably no more frequent in SLE when compared to the general population, while migraine with aura may be commoner in SLE sufferers especially with anticardiolipin antibodies. In children with SLE, headache may be associated with CNS involvement and may lead to referral for an MRI.
To summarize:- neurological dysfunction is an important cause of disability in SLE and many neurological disorders will prompt a neurologist to exclude the presence of a co-existing rheumatic condition such as SLE.
Raynauds Syndrome is another of the overlapping diseases that is autoimmune in nature and can develop either before or after a lupus diagnosis. Many will not develop it at all. Some will even have parts of it but not all. In the interest of discussion and education, I am posting this imformation from the www.mayoclinic.com website.
*It is important to note that you can have Raynauds and not have lupus. If you suspect you may have it, please seek medical attention*
By Mayo Clinic staff
Raynaud’s disease is a condition that causes some areas of your body — such as your fingers, toes, tip of your nose and your ears — to feel numb and cool in response to cold temperatures or stress. In Raynaud’s disease, smaller arteries that supply blood to your skin narrow, limiting blood circulation to affected areas.
Women are more likely to have Raynaud’s disease. It’s also more common in people who live in colder climates.
Treatment of Raynaud’s disease depends on its severity and the presence of associated conditions. For most people, Raynaud’s disease is more a nuisance than a disability.
By Mayo Clinic staff
Raynaud’s disease is more than simply having cold hands and cold feet, and it’s not the same as frostbite. Signs and symptoms of Raynaud’s depend on the frequency, duration and severity of the blood vessel spasms that underlie the disorder. Raynaud’s disease symptoms include:
- Cold fingers and toes
- Sequence of color changes in your skin in response to cold or stress
- Numb, prickly feeling or stinging pain upon warming or relief of stress
During an attack of Raynaud’s, affected areas of your skin usually turn white at first. Then, the affected areas often turn blue, feel cold and numb, and your sensory perception is dulled. As circulation improves, the affected areas may turn red, throb, tingle or swell. The order of the changes of color isn’t the same for all people, and not everyone experiences all three colors.
Occasionally, an attack affects just one or two fingers or toes. Attacks don’t necessarily always affect the same digits. Although Raynaud’s most commonly affects your fingers and toes, the condition can also affect other areas of your body, such as your nose, lips, ears and even nipples. An attack may last less than a minute to several hours.
People who have Raynaud’s accompanied by another disease will likely also have signs and symptoms related to their basic underlying condition.
When to see a doctor
See your doctor right away if you have a history of severe Raynaud’s and develop an ulcer or infection in one of your affected fingers or toes.
By Mayo Clinic staff
Doctors don’t completely understand the cause of Raynaud’s attacks, but blood vessels in the hands and feet appear to overreact to cold temperatures or stress:
- Cold temperatures. When your body is exposed to cold temperatures, your extremities lose heat. Your body slows down blood supply to your fingers and toes to preserve your body’s core temperature. Your body specifically reduces blood flow by narrowing the small arteries under the skin of your extremities. In people with Raynaud’s, this normal response is exaggerated.
- Stress. Stress causes a similar reaction to cold in the body, and likewise the body’s response may be exaggerated in people with Raynaud’s.
Blood vessels in spasm
With Raynaud’s, arteries to your fingers and toes go into what’s called vasospasm. This narrows your vessels dramatically and temporarily limits blood supply. Over time, these same small arteries may also thicken slightly, further limiting blood flow. The result is that affected skin turns a pale and dusky color due to the lack of blood flow to the area. Once the spasms go away and blood returns to the area, the tissue may turn red before returning to a normal color.
Cold temperatures are most likely to trigger an attack. Exposure to cold can be as simple as putting your hands under a faucet of running cold water, taking something out of the freezer or exposure to cold air. For some people, exposure to cold temperatures isn’t necessary. Emotional stress alone can cause an episode of Raynaud’s.
Raynaud’s may be partly an inherited disorder.
Primary vs. secondary Raynaud’s
Raynaud’s occurs in two main types:
- Primary Raynaud’s. This is Raynaud’s without an underlying disease or associated medical problem that could provoke vasospasm. Also called Raynaud’s disease, it’s the most common form of the disorder.
- Secondary Raynaud’s. Also called Raynaud’s phenomenon, this form is caused by an underlying problem. Although secondary Raynaud’s is less common than the primary form, it tends to be a more serious disorder. Signs and symptoms of secondary Raynaud’s usually first appear at later ages — around 40 — than they do for people with the primary form of Raynaud’s.
Causes of secondary Raynaud’s include:
- Scleroderma. Raynaud’s phenomenon occurs in the majority of people who have scleroderma — a rare disease that leads to hardening and scarring of the skin. Scleroderma, a type of connective tissue disease, results in Raynaud’s because the disease reduces blood flow to the extremities.
- Lupus. Raynaud’s is also a common problem for people with lupus erythematosus — an autoimmune disease that can affect many parts of your body, including your skin, joints, organs and blood vessels. An autoimmune disease is one in which your immune system attacks healthy tissue.
- Rheumatoid arthritis. Raynaud’s may be an initial sign of rheumatoid arthritis — an inflammatory condition causing pain and stiffness in the joints, often including the hands and feet.
- Sjogren’s syndrome. Raynaud’s phenomenon can also occur in people who have Sjogren’s syndrome — an autoimmune disorder that may accompany scleroderma, lupus or rheumatoid arthritis.
- Diseases of the arteries. Raynaud’s phenomenon can be associated with various diseases that affect arteries, such as atherosclerosis, which is the gradual buildup of plaques in blood vessels that feed the heart (coronary arteries), or Buerger’s disease, a disorder in which the blood vessels of the hands and feet become inflamed. Primary pulmonary hypertension, a type of high blood pressure that affects the arteries of the lungs, can be associated with Raynaud’s.
- Carpal tunnel syndrome. The carpal tunnel is a narrow passageway in your wrist that protects a major nerve to your hand. Carpal tunnel syndrome is a condition in which pressure is put on this nerve, producing numbness and pain in the affected hand. The affected hand may become more susceptible to cold temperatures and episodes of Raynaud’s.
- Repetitive trauma. Raynaud’s can also be caused by repetitive trauma that damages nerves serving blood vessels in the hands and feet. Some people who type or play the piano vigorously or for long periods of time may be susceptible to Raynaud’s. Workers who operate vibrating tools can develop a type of Raynaud’s phenomenon called vibration-induced white finger.
- Smoking. Smoking constricts blood vessels and is a potential cause of Raynaud’s.
- Injuries. Prior injuries to the hands or feet, such as wrist fracture, surgery or frostbite, can lead to Raynaud’s phenomenon.
- Certain medications. Some drugs — including beta blockers, which are used to treat high blood pressure; migraine medications that contain ergotamine; medications containing estrogen; certain chemotherapy agents; and drugs that cause blood vessels to narrow, such as some over-the-counter cold medications — have been linked to Raynaud’s.
- Chemical exposure. People exposed to vinyl chloride, such as those who work in the plastics industry, may develop an illness similar to scleroderma. Raynaud’s can be a part of that illness.
- Other causes. Raynaud’s has also been linked to thyroid gland disorders.
By Mayo Clinic staff
Risk factors for primary Raynaud’s include:
- Your gender. Primary Raynaud’s affects women more than men.
- Your age. Although anyone can develop the condition, primary Raynaud’s often begins between the ages of 15 and 30.
- Where you live. The disorder is also more common in people who live in colder climates.
- Your family history. Additionally, a family history appears to increase your risk of primary Raynaud’s. About one-third of people with primary Raynaud’s have a first-degree relative — a parent, sibling or child — with the disorder.
Risk factors for secondary Raynaud’s include:
- Associated diseases. These include conditions such as scleroderma and lupus.
- Certain occupations. People in occupations that cause repetitive trauma, such as workers who operate tools that vibrate, also may be more vulnerable to secondary Raynaud’s.
- Exposure to certain substances. Smoking, medications that affect the blood vessels and exposure to chemicals such as vinyl chloride are associated with an increased risk of Raynaud’s.
By Mayo Clinic staff
If Raynaud’s is severe — which is rare — blood circulation to your fingers or toes could permanently diminish, causing deformities of your fingers or toes.
If an artery to an affected area becomes blocked completely, sores (skin ulcers) or dead tissue (gangrene) may develop. Ulcers and gangrene can be difficult to treat.
Tests and diagnosis
By Mayo Clinic staff
To diagnose Raynaud’s, your doctor will ask detailed questions about your symptoms and medical history and conduct a physical examination. Your doctor may also run tests to rule out other medical problems that may cause similar signs and symptoms, such as a pinched nerve.
Your doctor may perform a simple test called a cold-stimulation test during your office visit. This test may involve placing your hands in cool water or exposing you to cold air, to trigger an episode of Raynaud’s.
Sorting out primary vs. secondary Raynaud’s
To distinguish between primary and secondary Raynaud’s, your doctor may perform an in-office test called nail fold capillaroscopy. During the test, the doctor examines your nail fold — the skin at the base of your fingernail — under a microscope. Tiny blood vessels (capillaries) near the nail fold that are enlarged or deformed may indicate an underlying disease. However, some secondary diseases can’t be detected by this test.
If your doctor suspects that another condition, such as an autoimmune or connective tissue disease, underlies Raynaud’s, he or she may order blood tests, such as:
- Antinuclear antibodies test. A positive test for the presence of these antibodies — produced by your immune system — indicates a stimulated immune system and is common in people who have connective tissue diseases or other autoimmune disorders.
- Erythrocyte sedimentation rate. This blood test determines the rate at which red blood cells settle to the bottom of a tube in the space of an hour. A faster than normal rate may signal an underlying inflammatory or autoimmune disease. Autoimmune diseases are commonly associated with secondary Raynaud’s.
There’s no single blood test to diagnose Raynaud’s. Your doctor may order other tests, such as those that rule out diseases of the arteries, to help pinpoint a disease or condition that may be associated with Raynaud’s.
Treatments and drugs
By Mayo Clinic staff
Self-care and prevention steps usually are effective in dealing with mild symptoms of Raynaud’s. If these aren’t adequate, however, medications are available to treat more-severe forms of the condition. The goals of treatment are to:
- Reduce the number and severity of attacks
- Prevent tissue damage
- Treat any underlying disease or condition
Depending on the cause of your symptoms, medications may prove effective at treating Raynaud’s. To widen (dilate) blood vessels and promote circulation, your doctor may prescribe:
- Calcium channel blockers. These drugs relax and open up small blood vessels in your hands and feet. They decrease the frequency and severity of attacks in most people with Raynaud’s. These drugs can also help heal skin ulcers on your fingers or toes. Examples include nifedipine (Adalat CC, Afeditab CR, Procardia), amlodipine (Norvasc) and felodipine (Plendil).
- Alpha blockers. Some people find relief with drugs called alpha blockers, which counteract the actions of norepinephrine, a hormone that constricts blood vessels. Examples include prazosin (Minipress) and doxazosin (Cardura).
- Vasodilators. Some doctors prescribe a vasodilator — a drug that relaxes blood vessels — such as nitroglycerin cream to your fingers to help heal skin ulcers. Your doctor may also prescribe vasodilator drugs that are commonly used to treat other conditions, but may effectively relieve the symptoms of Raynaud’s. These drugs include the high blood pressure drug losartan (Cozaar), the erectile dysfunction medication sildenafil (Viagra), the antidepressant medication fluoxetine (Prozac), and a class of medication called prostaglandins.
You and your doctor may find that one drug works better for you than another. Some drugs used to treat Raynaud’s have side effects that may require you to stop taking the medication. A drug may also lose effectiveness over time. Work with your doctor to find what works best for you.
Some medications actually can aggravate Raynaud’s by leading to increased blood vessel spasm. Your doctor may recommend that you avoid taking:
- Certain over-the-counter (OTC) cold drugs. Examples include drugs that contain pseudoephedrine (Actifed, Chlor-Trimeton, Sudafed).
- Beta blockers. This class of drugs, used to treat high blood pressure and heart disease, includes metoprolol (Lopressor, Toprol), nadolol (Corgard) and propranolol (Inderal, Innopran XL).
- Birth control pills. If you use birth control pills, you may wish to switch to another method of contraception because these drugs affect your circulation and may make you more prone to attacks. Talk to your doctor before stopping the pill.
If you have questions about how best to manage Raynaud’s, contact your doctor. Your primary care doctor may refer you to a physician who specializes in treating Raynaud’s.
Sometimes in cases of severe Raynaud’s, approaches other than medications may be a treatment option:
- Nerve surgery. Nerves called sympathetic nerves in your hands and feet control the opening and narrowing of blood vessels in your skin. Sometimes it’s necessary in cases of severe Raynaud’s to cut these nerves to interrupt their exaggerated response. Through small incisions in the affected hands or feet, a doctor strips away these tiny nerves around the blood vessels. The surgery, called sympathectomy, may reduce the frequency and duration of attacks, but it’s not always successful.
- Chemical injection. Doctors can inject chemicals to block sympathetic nerves in affected hands or feet. You may need to have the procedure repeated if symptoms return or persist.
- Amputation. Sometimes, doctors need to remove tissue damaged from a lack of blood supply. This may include amputating a finger or toe affected by Raynaud’s in which the blood supply has been completely blocked and the tissue has developed gangrene. But this is rare.
Lifestyle and home remedies
By Mayo Clinic staff
A variety of steps can decrease Raynaud’s attacks and help you feel better overall:
- Don’t smoke. Smoking causes skin temperature to drop by constricting blood vessels, which may lead to an attack. Inhaling secondhand smoke also may aggravate Raynaud’s.
- Exercise. Your doctor may encourage you to exercise regularly, particularly if you have primary Raynaud’s. Exercise can increase circulation, among other health benefits.
- Control stress. Because stress may trigger an attack, learning to recognize and avoid stressful situations may help control the number of attacks.
- Avoid caffeine. Caffeine causes your blood vessels to narrow and may increase the signs and symptoms of Raynaud’s.
- Take care of your hands and feet. If you have Raynaud’s, guard your hands and feet from injury. Don’t walk barefoot. Take care of your nails to avoid injuring sensitive toes and fingertips. In addition, avoid wearing anything that compresses blood vessels in your hands or feet, such as tight wristbands, rings or footwear.
- Avoid workplace triggers. Avoiding tools that vibrate the hand may reduce the frequency of attacks.
During an attack: What should you do?
What should you do if you’re experiencing an attack of Raynaud’s? The first and most important action is to warm your hands or feet or any other affected areas of skin. The following steps can help you gently warm your fingers and toes:
- Move to a warmer area.
- Place your hands under your armpits.
- Wiggle your fingers and toes.
- Make wide circles, or windmills, with your arms.
- Run warm — but not hot — water over your fingers and toes.
- Massage your hands and feet.
If a stressful situation triggers an attack, you can help stop the attack by getting out of the stressful situation and relaxing. If you’re trained in biofeedback, you can use this technique along with warming your hands or feet in water to help lessen the attack.
Sjogrens (pronounced show grins) syndrome is an autoimmune disease which sometimes overlaps with lupus. It can cause a myriad of issues and is not well known by the public. In an effort to educate, I am presenting this article, taken form the www.sjogrens.org website. Please read and learn. Thanks!
Sjögren’s syndrome is a chronic autoimmune disease in which people’s white blood cells attack their moisture-producing glands. Today, as many as four million Americans are living with this disease.
Although the hallmark symptoms are dry eyes and dry mouth, Sjögren’s may also cause dysfunction of other organs such as the kidneys, gastrointestinal system, blood vessels, lungs, liver, pancreas, and the central nervous system. Patients may also experience extreme fatigue and joint pain and have a higher risk of developing lymphoma.
With upwards of 4,000,000 Americans suffering from Sjögren’s syndrome, it is one of the most prevalent autoimmune disorders. Nine out of 10 patients are women.
About half of the time Sjögren’s syndrome occurs alone, and the other half it occurs in the presence of another autoimmune connective tissue disease such as rheumatoid arthritis, lupus, or scleroderma. When Sjögren’s occurs alone, it is referred to as “Primary Sjögren’s.” When it occurs with another connective tissue disease, it is referred to as “Secondary Sjögren’s.”
All instances of Sjögren’s syndrome are systemic, affecting the entire body. Symptoms may remain steady, worsen, or, uncommonly, go into remission. While some people experience mild discomfort, others suffer debilitating symptoms that greatly impair their functioning. Early diagnosis and proper treatment are important — they may prevent serious complications and greatly improve a patient’s quality of life.
Since symptoms of Sjögren’s syndrome mimic other conditions and diseases, Sjögren’s can often be overlooked or misdiagnosed. On average, it takes nearly seven years to receive a diagnosis of Sjögren’s syndrome. Patients need to remember to be pro-active in talking with their physicians and dentists about their symptoms and potential treatment options.
Since the disease was first identified in 1933 by Dr. Henrik Sjögren, it has been proven to affect virtually every racial and ethnic group. General awareness about Sjögren’s syndrome is still lacking and increased professional awareness is needed to help expedite new diagnoses and treatment options.
Sjögren’s syndrome is a systemic disease, and its symptoms are felt throughout the entire body.
Symptoms vary from person to person but may include:
- a dry, gritty or burning sensation in the eyes
- dry mouth
- difficulty talking, chewing or swallowing
- a sore or cracked tongue
- dry or burning throat
- dry or peeling lips
- a change in taste or smell
- increased dental decay
- joint pain
- vaginal and skin dryness
- digestive problems
- dry nose
Early diagnosis and treatment are important for preventing complications. Unfortunately, reaching a diagnosis can often be difficult and has been found to take an average of 6.5 years from the onset of symptoms.
Sjögren’s syndrome symptoms frequently overlap with or “mimic” those of other diseases including lupus, rheumatoid arthritis, fibromyalgia, chronic fatigue syndrome, and multiple sclerosis. Dryness can also occur for other reasons, such as a side effect of medications such as anti-depressants and high blood pressure medication.
There is no single test that will confirm diagnosis. Rheumatologists have primary responsibility for diagnosing and managing Sjögren’s syndrome and can conduct a series of tests and ask about symptoms. An international group of experts formulated classification criteria for Sjögren’s syndrome which help doctors arrive at a diagnosis. These criteria consider dryness symptoms, changes in salivary (mouth) and lacrimal (eye) gland function, and systemic (whole body) findings.
Blood tests your physician may perform include:
- ANA (Anti-Nuclear Antibody)
ANAs are a group of antibodies that react against normal components of a cell nucleus. About 70% of Sjögren’s patients have a positive ANA test result.
- RF (Rheumatoid Factor)
This antibody test is indicative of a most often performed for the diagnosis of rheumatoid arthritis (RA) but is positive in many rheumatic diseases. In Sjögren’s patients, 60-70% have a positive RF.
- SS-A (or Ro) and SS-B (or La)
These are the marker antibodies for Sjögren’s. Seventy percent of Sjögren’s patients are positive for SS-A and 40% are positive for SS-B (these may also found in lupus patients).
- ESR (Erythrocyte Sedimentation Rate)
This test measures inflammation. An elevated ESR indicates the presence of an inflammatory disorder, including Sjögren’s syndrome.
- IGs (Immunoglobulins)
These are normal blood proteins that participate in immune reactions and are usually elevated in Sjögren’s patients.
The ophthalmologic (eye) tests include:
- Schirmer Test
Measures tear production.
- Rose Bengal and Lissamine Green
Eyedrops containing dyes that an eye care specialist uses to examine the surface of the eye for dry spots.
The dental tests include:
- Salivary Flow
Measures the amount of saliva produced over a certain period of time.
- Salivary scintigraphy
A nuclear medicine test that measures salivary gland function.
- Salivary gland biopsy (usually in the lower lip)
Confirms inflammatory cell (lymphocytic) infiltration of the minor salivary glands.
Your physician will consider the results of these tests along with your physical examination to arrive at a final diagnosis. Further research is being conducted to refine the diagnostic criteria for Sjögren’s syndrome and to help make diagnosis easier and more accurate.
Early diagnosis and high-quality professional care are extremely important for Sjögren’s patients.
Currently, there is no cure for Sjögren’s syndrome. However, treatments may improve various symptoms and prevent complications.
In addition to over the counter (OTC) eye drops and mouth preparations, prescription products for dry eyes and dry mouth are available. They include Evoxac® (cevimeline), Salagen® (pilocarpine hydrochloride) and Numoisyn™ for dry mouth and Restasis® (cyclosporine ophthalmic emulsion) and Lacrisert® (hydroxypropyl cellulose ophthalmic insert) for dry eye.
Some patients are prescribed immunosuppressive medications to treat their internal organ manifestations. Physicians may also prescribe other medications for systemic manifestations or severe flares. Since Sjögren’s syndrome affects each patient differently, a personalized plan should be developed by you and your physician, dentist and eye care specialist about how to treat your various symptoms.
In addition, many symptoms and problems of Sjögren’s syndrome can be treated with over-the-counter medications. These medications can help to alleviate different types of dryness and pain, but you should check with your physician when adding these medications to your regimen. He/she may have suggestions for what products you should use and may also give you some tips on how and when to use them.
For a listing of these and additional treatment options available, a Product Directory is available to Foundation members in the Member Community section of our website.
I have noticed in my own experience and others, that when we are diagnosed with one autoimmune disease, sometimes we develop more that will overlap with our original diagnosis. For instance, I have SLE or lupus, and overlapping that I have fibromyalgia, IBS, corneal erosion, raynauds and a host of other autoimmune diseases. I found this information on the website, www.lupus.org from the Lupus Foundation of America. It makes an interesting read…
Lupus and Overlap
Connective Tissue Disease And Overlap Syndromes
The connective tissue diseases are a family of closely related disorders. They include: rheumatoid arthritis (RA), systemic lupus erythematosus (SLE or lupus), polymyositis-dermatomyositis (PM-DM), systemic sclerosis (SSc or scleroderma), Sjogren’s syndrome (SS) and various forms of vasculitis.
These diseases have a number of common features:
- They affect women much more frequently than men.
- They are “multisystem” diseases, capable of affecting the function of many organs.
- They “overlap” with one another, sharing certain clinical symptoms, signs, and laboratory abnormalities.
- Blood vessels are the most common target of injury in all of these diseases.
- The immune system is abnormal and accounts, at least in part, for the observed tissue damage.
Although lupus most often occurs alone, many people with lupus also have symptoms characteristic of one or more of the other connective tissue diseases. In this circumstance, a physician may use the term “overlap” to describe the illness. There are several well-recognized overlaps that may affect people with lupus.
Lupus and Rheumatoid Arthritis
In lupus, joint pain (arthralgia) is common. Joint swelling (arthritis) may be present in some cases, but the majority of those with lupus experience joint pain without swelling or only intermittent swelling. In rheumatoid arthritis (RA), joint swelling is always present and pain is common but less prominent. Because rheumatoid arthritis is more likely than lupus to cause joint deformities and bone destruction, joint replacement or reconstructive surgery is more often required in RA than in SLE. If a person with lupus develops severe arthritis with joint deformities, he/she should be considered to have rheumatoid-like arthritis. In some instances, the physician might have reason to believe that both diseases — SLE and RA — have occurred in the same person. When arthritis develops in the course of lupus, treatment with non-steroidal anti-inflammatory drugs (NSAIDs), low doses of cortisone, and the antimalarial drug hydroxychloroquine (Plaquenil) are usually helpful. People with lupus who have typical rheumatoid arthritis are prescribed the standard forms of RA treatment. These include methotrexate, sulfasalazine and in some cases, more potent drugs to suppress joint inflammation.
Many persons with lupus have muscle pain (myalgia), but a few have muscle weakness due to inflammation (myositis). The “muscle weakness” that people with lupus report is most commonly due to fatigue or high doses of cortisone. In polymyositis-dermatomyositis, the primary problem is muscle weakness due to muscle inflammation. In myositis, weakness especially affects the hips (inability to rise from a chair or toilet seat, or to climb stairs unassisted) and shoulders (inability to lift a weight onto a high shelf or comb one’s hair). Typically, there is little or no pain associated with the weakness. People with myositis have increased blood levels of creatine kinase (CK, a substance that leaks from injured muscle), abnormal electrical activity of muscles (seen in an electromyogram, or EMG), and muscle cell degeneration and inflammation that is found in a muscle biopsy. Prednisone or other cortisone-like drugs are most often recommended for the treatment of myositis, and may be used in combination with other immune-suppressing drugs. Cortisone itself, in high doses, may actually cause muscle weakness of the hips and shoulders, very similar to what occurs in myositis. But in this condition, called “steroid myopathy,” the CK, EMG, and the muscle biopsy do not suggest inflammation, and recovery of strength promptly follows reduction of the cortisone dose.
Lupus and Scleroderma
The hallmark of scleroderma (SSc) is thickened skin (sclero=hard, derma=skin) which affects the fingers, and often the hands, forearms, feet, and face. If skin thickening is widespread, it may extend to the upper arms, thighs, chest, and abdomen. These changes are due to the excessive production and uncontrolled laydown of collagen, the substance normally present in scar tissue. The variety of skin rashes seen in lupus are due to inflammation, rather than fibrosis. These include the facial “butterfly” rash and photosensitivity reaction (rash, hives or blisters immediately after exposure to sunlight or other sources of ultraviolet light). The latter is limited to the skin surfaces exposed. An exception is discoid lupus, which consists of spots or patches of rash, mostly in sun exposed areas (face, ears, extremities), which typically cause scarring and skin pigment changes. The appearance of scleroderma and discoid lupus are entirely different, and should be easily distinguished from one another by your physician.
Other features less common in SLE than in SSc include: scarring of the lower portions of the lung (pulmonary fibrosis); difficulty in swallowing solid foods such as bread or meat; and heartburn or indigestion from stomach acid “refluxing” (coming back up) into the esophagus. Difficulty swallowing and reflux are due to sluggish and uncoordinated motion of the muscle layer of the esophagus (esophageal dysmotility). Scleroderma often leads to finger and hand deformities as well, due to the combination of skin thickening, arthritis, tendinitis and tenosynovitis (inflammation and scarring of tendons and their lining tissues). These processes ultimately result in limited movement of the fingers. Raynaud’s phenomenon — when fingers turn blue or white with cold — occurs in 95 percent of people with scleroderma and in 40 percent of persons with lupus.
The primary treatment approaches to SSc are quite different from those for lupus. Therefore, treatment for scleroderma-like problems in people with lupus should be individualized and directed at the particular problems present at any given time.
Lupus and Overlap
Mixed Connective Tissue Disease
Some individuals have symptoms and signs of three connective tissue diseases, i.e., lupus, polymyositis-dermatomyositis, and scleroderma. At any given time, the combination of problems encountered by the patient may vary considerably, from no active disease to features of one, two, or all three of these conditions at the same time. These persons often (but not always) have one specific blood antibody in their blood (anti-U1RNP antibody) but not the other antibodies commonly associated with SLE, SSc, or PM-DM. Whether this is an entirely separate disease, or a situation in which one person has three diseases, remains uncertain. However, the presence of a single blood antibody is a strong point in favor of a distinctive disease. As in the other circumstances mentioned above, treatment should be individualized and directed at the particular problems present at any given time.
Henrik Sjögren was a Swedish ophthalmologist and the first to recognize that dry eyes and dry mouth were often found in people with connective tissue diseases. These symptoms are caused by the accumulation of immune system cells (lymphocytes) in and around tear and saliva producing glands. The build-up of cells disturbs the function of these glands and leads to reduced production of tears and saliva. This condition also interferes with the protective mechanisms of the eye and mouth. Eye inflammation and ulcers of the cornea, as well as fungal infections of the mouth (thrush), occur with increased frequency in those with Sjogren’s. Rarely, a person with this disorder develops a malignancy (cancer) affecting the lymphocytes (lymphoma). Today, Sjogren’s syndrome is itself accorded the status of a distinct connective tissue disorder.
Sjogren’s Syndrome also occurs in some people with lupus. They have an increased frequency of sun-sensitive rashes and Sjogren’s-related blood antibodies (anti-SSA and anti-SSB antibodies). Women with anti-SSA antibodies are at increased risk of having babies with “neonatal lupus.” Symptoms in the infant can be as minor as a temporary lupus-like skin rash, or as serious as permanent damage to the electrical system of the heart which results in a very slow heart rate (complete heart block).
The best treatments for Sjogren’s Syndrome include: artificial tears (usually satisfactory) and either artificial saliva (most often unsatisfactory) or a saliva stimulant such as pilocarpine, and hydroxychloroquine (Plaquenil). Eye drops containing cyclosporin have also just been introduced and have significant benefit for dry eyes in some cases. Arthritis, fatigue and skin rash in people with Sjogren’s is often treated with Plaquenil.
Frequency Of Overlap Syndromes In People With Lupus
The majority of people with lupus have lupus alone. Between 5 and 30 percent of people with lupus report having overlap symptoms. The likelihood of a person with lupus also having an overlap disease is 15 percent, distributed as follows:
|Mixed Connective Tissue Disease
Heredity And Overlap
It is unusual (less than 10 percent of the time) for a person with lupus to have a close family member (parent, child, brother, or sister) who also suffers from lupus. However, it is common for persons with lupus to have relatives (including grandparents, aunts/uncles, cousins) with other connective tissue diseases such as rheumatoid arthritis, Sjogren’s Syndrome, scleroderma, etc. These co-occurrences raise the possibility that heredity may be a factor in all of the connective tissue diseases. Most scientists agree that important hereditary associations with these diseases are present in some families. Additional research is needed to shed light on this important question.
Prognosis For People With Lupus And Overlap Syndromes
It is important for those with lupus to be aware of the symptoms that might indicate the development of “overlap” features, since these symptoms and abnormalities may be best managed with treatments not typically used for lupus. Fortunately, when an overlap syndrome is present, the symptoms characteristic of the other connective tissue diseases involved are usually mild and not life-threatening.
As I woke up this morning, I was wondering what the day would bring. I felt good and went about getting some laundry done and dishes done and other mundane household chores. I was feeling great so I enjoy doing these things when I feel great.
As the day has gone on, I have found my strength waning, and my energy almost all gone. It is like someone has a lightswitch and when it is “on” I am fine (at least for me, lol). The change of energy can occur much like that light switch, and as quickly. I was humming around the house one minute and then in a few more minutes, I was in bed. Yes, in bed. Arms and legs feel like spaghetti noodles, and pain has increased as day has gone on. I took my meds and yet it still hurts.
As I am laying here I noticed that my kitty cat, Shelby, has joined me and is sleeping next to me. If you have read any of my previous posts, you will know how she seems to sense that I need comfort and she stays next to me when I am not feeling good. When I got up and went into the kitchen, she followed. When I came back to bed, she followed. She seems to feel it is her duty to make sure I am ok and if she has to stay by my side, that is what she does. It is amazing to see how intuitive animals can be.
Our dog is even in here with me. I am not sure if she is intuitive, but she wants company so here she is too.
So, in a nutshell, I got the day started off well, then it deteriorated later in the day to where I am now in bed, resting. Have I mentioned how much I hate lupus? It can take a great day and turn it upside down in a matter of minutes, it seems, and make me a slave to the bed. So, I am resting here in bed, playing computer games, and napping off and on. That is how you attempt to tame the beast. If you do not listen to your body, you will get worse, not better.
My Walk for Lupus Now event is Saturday at 9 am. I am hoping to get to walk, but we will see how I feel. I can attend and not walk being as I am a lupus patient, and it doesn’t matter one way or the other. I liked walking last year and walked a mile! woot woot! Last year our team raised $125 and I set our goal this year at $250. Sadly, we will not make it, it looks like, but we have beaten last years total so that is a good thing. It counts!
Several of my walkers have been hit hard with difficulties. One has broken her foot, another tore a tendon and found out her husband has prostate cancer, another one’s husband passed away a few days ago, another one got a new job and has to work, and so it leaves just me, my 5 year old granddaughter, and my daughter in law and other granddaughter to walk and represent our team. Our team name is Beautiful Butterflies. I am the captain. This is only our second year having this walk so I am pleased that it is getting the attention it deserves.
Next year, I will work harder to get some corporate sponsorship and giveaways and raffles and such to earn more money to fund the research we so desperately need for this disease. I, personally, am sick and tired of being sick and tired and I worry about my grandchildren and children developing this disease as a result of genetics and me passing it on to them. I pray that doesn’t happen but you never know. That is my reason for walking, to find a cure. A cure will keep them all safe from the threat of a life in pan and uncertainty. It gives me hope to think it may happen.
Well, I feel a little nappie coming on so that is it for now. I am hoping I can research some of those other autoimmune diseases for a future blog post. I found it interesting to see the list and read a bit about each one. I think others may like that as well. Thanks to you all for reading this blog. I truly appreciate each and every one of you!
These medications will vary from person to person because lupus is different in each individual and only your doctor can decide what is the best line of treatment for your symptoms. This is just an overall guide. Some people will never have to take most of these drugs while others may have to take most of them at some point in their disease. This information is from the Lupus Foundation of America’s web page. Please refer to their page for more information.
Medications to Treat Lupus Symptoms
Anti-inflammatory medications help to relieve many of the symptoms of lupus by reducing inflammation and pain. Anti-inflammatories are the most common drugs used to treat lupus, particularly symptoms such as fever, arthritis or pleurisy, which generally improve within several days of beginning treatment. For many people with lupus, an anti-inflammatory drug may be the only medication they need to control their lupus.
- Aspirin is inexpensive and available over the counter. It has pain-reducing, anti-inflammatory, and anticoagulant (blood-thinning) properties that can control some of the symptoms of lupus. However, aspirin can cause stomach irritation.
- Acetaminophen, known to most people as Tylenol®, is also used to reduce pain. Although it causes less stomach irritation than aspirin, acetaminophen does not help with inflammation and cannot control any of the disease activity of lupus. Most people have no side effects when taking Tylenol, but in rare cases acute liver failure has occurred.
- Non-Steroidal Anti-Inflammatory Drugs (NSAIDs) suppress inflammation and are especially useful for joint pain and stiffness. Examples of NSAIDs are ibuprofen (Motrin®), naproxen (Naprosyn®), indomethacin (Indocin®), nabumetone (Relafen®), and celecoxib (Celebrex®). People often respond better to one particular NSAID than another, so you may need to try several different products to determine the most effective one for you.
Like aspirin, NSAIDs can cause stomach irritation. NSAIDs may also cause serious gastrointestinal (GI) complications, such as a bleeding ulcer. To reduce the chance of these problems, NSAIDs are usually taken with food, milk or antacids, or may be accompanied by other medications such as misoprostol (Cytotec®), omeprazole (Prilosec®), lanzoprazole (Prevacid®), and others.
Side effects of NSAIDS, such as abnormal urine test results, occasionally may be mistaken for signs of active lupus. Recognizing this possible complication of NSAID use is important, because the symptoms will go away when the drug is stopped. In general, you should always be cautious about taking too much of any NSAID, as excessive amounts can reduce the blood flow to your kidneys and may interfere with their ability to remove waste from your body.
Corticosteroids (also known as glucocorticoids, cortisone or steroids) are synthetic (man-made) drugs designed to work like the body’s naturally occurring hormones produced by the adrenal glands, in particular cortisol. Hormones are the body’s chemical messengers that regulate most of the body’s functions. Cortisol helps regulate blood pressure and the immune system, and it is the body’s most potent anti-inflammatory hormone. Corticosteroids prescribed for autoimmune diseases are different from the anabolic steroids that weightlifters and other athletes sometimes take to increase strength.
Steroid medications work quickly to decrease the swelling, warmth, tenderness, and pain that are associated with inflammation. They do this by lessening the immune system’s response. Prednisone is the most commonly prescribed steroid for lupus. Prednisolone and methyl-prednisolone (Medrol®) are similar to prednisone, and some physicians prefer to prescribe these if you have liver problems.
Most people take steroids in pill form, but topical creams or gels are often used for cutaneous (skin) lupus. Steroids in liquid form are sometimes injected into muscles or directly into joints, and in some cases into skin lesions. Pulse steroids are large liquid doses given intravenously (injected into a vein) over several hours; the beneficial effects can last for weeks so pulse steroids are sometimes prescribed to control a lupus flare, or for people who cannot tolerate steroids in pill form.
Your doctor will try to keep your steroid dosage at the lowest effective level. Once the symptoms of lupus have responded to treatment, the steroid dose is gradually reduced (tapered). As an alternative to tapering, or stepping down the steroid dose, your doctor may choose to have you take steroids on an every-other day basis — one day on, one day off.
Steroids can produce a variety of side effects. The most common are changes in appearance (acne, a round or moon-shaped face, weight gain due to increased appetite, and hair growth). Steroids can cause fluid retention and a redistribution of fat, leading to a swollen face and abdomen, but thin arms and legs. Also, the skin becomes more fragile and bruises easily. Steroids can suppress growth in children. Steroids can also cause irritability, agitation, excitability, insomnia, or depression. These changes in appearance and mood are more apparent with high doses of steroids.
Side Effects of Long Term Steroid Use
- Increased risk of infections poses the most danger. If you are taking steroids you must take extra care to clean and protect any open wounds. Infections are one of the leading causes of death in people with lupus.
- Avascular necrosis of bone, which occurs most often in the hip, is the destruction of the bone itself and is quite painful. Relief from pain often requires total surgical joint replacement.
- Osteoporosis (bones become fragile and more likely to break) leads to bone fractures, especially compression fractures of the vertebrae with severe back pain.
- Muscle weakness
- Suppression of growth in children
Antimalarials are used in combination with steroids and other medications, in part to reduce the dose required of the other drugs. Antimalarials are most often prescribed for skin rashes, mouth ulcers, and joint pain, but also can be effective in mild forms of lupus where inflammation and blood clotting are a concern. Antimalarials improve lupus by decreasing autoantibody production, protecting against the damaging effects of ultraviolet light from the sun and other sources, and improving skin lesions.
The two types of antimalarials most often prescribed today for lupus are hydroxychloroquine (Plaquenil®) and chloroquine (Aralen®). Unlike the rapid response seen with steroids, it may take months before antimalarial drugs improve your lupus symptoms.
Side effects from antimalarials are rare and usually mild; they include upset stomach and changes in skin color. These side effects usually go away after the body adjusts to the medication. In high doses certain antimalarial drugs may damage the retina of the eye, causing vision problems. With the low doses of antimalarials used in the treatment of lupus, the risk of this complication is extremely low. However, as a precaution, people treated with antimalarials should see an eye doctor (ophthalmologist) regularly.
Women who are pregnant should continue to take their antimalarial medication as prescribed, in order to avoid a lupus flare. Although this medication can cross the placenta, the possibility of eye and ear toxicity in the infant is very low. In fact, recent studies suggest that the risk of flare for the mother is greater than the risk of fetal toxicity.
Immunosuppressives (Immune Modulators)
Immunosuppressive medications are used to control inflammation and the overactive immune system, especially when steroids have been unable to bring lupus symptoms under control, or when a person cannot tolerate high doses of steroids. However, there can be serious side effects from these drugs, so if you are being treated with immunosuppressives you should be carefully monitored by your physician. Immunosuppressive drugs reduce your body’s ability to fight off infections, and increase the chances that you could develop viral infections such as shingles (chicken pox, or herpes zoster). It is extremely important that you pay attention to even the smallest cut or wound, and let your doctor know if any sign of infection begins, such as redness, swelling, tenderness, or pain. These drugs may also increase your risk for developing cancer.
Each immunosuppressive drug has unique side effects. Therefore it is important that immunosuppressive drugs be given only by physicians who are experienced with the use of these medications.
Cyclophosphamide (Cytoxan®) was developed to fight cancer. Although in its early years of use it was taken in pill form, today Cytoxan is taken through the vein (intravenously, or IV). It has been shown to improve kidney and lung disease, but can affect a woman’s menstrual cycle and can cause bladder problems, hair loss, and sterility.
Methotrexate (Rheumatrex™), also developed to fight cancer, is known as the “gold standard” — the best drug — for the treatment of rheumatoid arthritis. It has also been shown to be very effective in treating skin lesions, arthritis, and pleuritis in people with lupus. However, the drug can cause sun-sensitivity, liver damage, including cirrhosis, and lung infections. If you are taking this drug you should not drink alcohol, especially if you have a history of kidney disease. If you are taking high-dose methotrexate you should not use NSAIDs; caution is also advised when taking aspirin. Nausea, mouth sores, and headaches are the most common side effects of methotrexate.
Azathioprine (Imuran®) was developed to prevent rejection of kidney transplants. It blocks inflammation pathways in lupus and helps to lower the steroid dosage and improve liver and kidney disease. However, it may cause pancreatitis and an allergic form of hepatitis, so liver function tests and blood counts should be done regularly.
Because blood clots can be a life-threatening symptom of lupus, these drugs are used to thin your blood to prevent it from clotting too easily. Anticoagulant medications include low-dose aspirin, heparin (Calciparine®, Liquaemin®) and warfarin (Coumadin®). In particular, if you are being treated with warfarin you must be monitored by your doctor to be sure your blood does not become too thin. Anticoagulant therapy may be lifelong in some people with lupus. Very recent research has shown that people’s genetic makeup may influence how they respond to warfarin; specifically, that people with variations in two genes may need lower warfarin doses due to differences in how the body breaks down (metabolizes) warfarin and regulates the ability of warfarin to prevent blood from clotting. Therefore the dosage and administration of warfarin must be individualized for each person.
Frequently Asked Questions
I don’t want to go on prednisone. Are there any other treatments available?
In addition to corticosteroids, lupus can be treated with non-steroidal anti-inflammatory drugs, anti-malarial medications, and chemotherapy drugs. There can be situations where steroids are the best choice of therapy and the other medications are not indicated or are ineffective.
What side effects can I expect from taking steroids?
Prednisone is a double-edged sword. It is a very effective anti-inflammatory agent in lupus, and it works fast. But over time, the side effects of higher doses of the medication can be significant. People taking steroids may have side effects that include weight gain (especially in the cheeks and over the back of the neck), acne, hair thinning on the scalp, new facial hair (on the chin or above the lips), mood swings and difficulty concentrating. Your doctor may also discover that your prednisone has caused higher blood pressure, higher glucose levels and higher cholesterol. Prednisone can also weaken bones and damage the blood supply to joints, which usually occurs first in the hips.
Does long-term prednisone use cause diabetes?
Cortisone and its analogues are “stress hormones” that prime the body for times of challenge. Thus, the rise in sugar in the body is a natural byproduct of a preparation for stress in tissues of the muscles, brain, and heart for example. This is why an increase in the stress hormone results in an increase of the body’s stores of glucose. Long-term prednisone use can cause diabetes in someone who has a tendency to be diabetic. Moreover, the higher the dose of prednisone, the greater the likelihood that the blood glucose (sugar) level will rise. Obesity and a genetic background that includes diabetes also gives a person a greater chance of developing diabetes.