Since the beast of fibromyalgia has reared its ugly head in my body recently, I decided to review the basic facts of fibromyalgia (fm). I always feel that knowledge is more powerful than ignorance so in my quest for the information, I found this website which has a great description of the basic information of fm. I got this from the website www.prohealth.com. You can find more information there. Here is their article:
What is Fibromyalgia?
Fibromyalgia (FM) is a chronic pain disorder characterized by widespread pain, fatigue and sleep disturbances. It was originally thought to be a musculoskeletal disorder since most of the pain was felt in the muscles and other soft tissues. However, recent research and the advancement of brain-imaging technology is revealing that fibromyalgia is actually a disorder of the central nervous system, which causes abnormal pain processing and results in pain amplification.
The three primary symptoms that are common to almost everyone with fibromyalgia are pain, fatigue and sleep disorders.
Pain: Pain is usually the most prominent symptom of fibromyalgia. FM pain is chronic and widespread, affecting all four quadrants of the body, although not necessarily at the same time. Its intensity may range from mild to profound. FM pain tends to migrate, sometimes affecting one part of the body and sometimes another. Patients also report that their bodies ache all over, much like having the flu. In addition to the aching, FM pain has been described by different people as burning, throbbing, sharp, stabbing or shooting pain. Most people with FM also complain of feeling stiff and achy when they wake up.
Fatigue: While everyone knows what it feels like to be tired, the fatigue experienced by fibromyalgia patients is so much more. It’s a pervasive, all-encompassing exhaustion that can interfere with even the most basic and simple daily activities. Another feature of FM fatigue is that it is not relieved by rest.
Sleep Disorders: Most people with fibromyalgia have problems with sleep. They report having difficulty getting to sleep, waking up frequently throughout the night, and not feeling refreshed when they get up in the morning. Studies have shown that FM patients spend little to no time in deep, stage four sleep because their sleep is repeatedly interrupted by bursts of awake-like brain activity. Since deep sleep is the time during which the body replenishes itself, fibromyalgia patients are not able to get the restorative sleep their bodies need. In turn, this lack of deep sleep can result in making their pain and fatigue worse.
Other Symptoms: Most fibromyalgia patients also report a number of other symptoms and overlapping conditions, which may include allergies, irritable bowel, irritable bladder, headaches, migraines, dizziness, numbness and tingling, sensitivity to cold or heat, depression, restless legs syndrome, chemical or environmental sensitivities, impaired balance or coordination, dry eyes and mouth, vision problems, or problems with memory, concentration and cognitive functioning.
(For more information about specific symptoms, see “Common Symptoms“)
Who is at Risk for Developing Fibromyalgia?
Adult women appear to be at greater risk for developing fibromyalgia than men or children, however, it can affect all ages and both sexes. Historically, 75 to 90 percent of people diagnosed with FM have been women, but new information may eventually change those figures.
FM experts are finding that men often have fewer than the traditional 11 tender points required for diagnosis, yet meet all the other criteria for fibromyalgia. And what was once thought to be “growing pains” in children may turn out to be a previously unrecognized form of FM.
Although fibromyalgia will probably still occur most frequently in adult women, we may soon discover it affects significantly more men and children than once thought.
Another risk factor may be family history, as there is growing evidence of a genetic component in fibromyalgia. If someone in your family has FM, you may be at greater risk of developing it yourself.
How is Fibromyalgia Diagnosed?
Fibromyalgia (FM) should be diagnosed by a qualified physician who is familiar with the illness and has experience diagnosing and treating it. In 1990, the American College of Rheumatology established the research criteria for fibromyalgia, which are now the criteria most commonly used to diagnose it.
For a diagnosis of fibromyalgia, both of the following must be present:
- A history of widespread pain in all four quadrants of the body for a minimum duration of three months, and
- Pain in at least 11 of 18 designated tender points when four kilograms (about 10 pounds) of pressure are applied.
According to the American College of Rheumatology fibromyalgia is not a diagnosis of exclusion. However, there are several other illnesses that have similar symptoms, so it is important that the doctor test for certain comorbid conditions as well. Which particular tests may be necessary will depend upon the individual patient’s symptoms and medical history.
In the past few years scientists have made tremendous progress unraveling the mysteries of fibromyalgia. Although there is still some disagreement as to the cause of FM, there is little disagreement that its onset is usually triggered by some form of trauma. The trauma may be physical, in the form of an injury or illness, or it could be an emotional trauma that produces severe, prolonged stress.
Current research seems to indicate that some people have a genetic predisposition to fibromyalgia, although the symptoms usually do not show up until triggered by one of these traumas.
One popular theory as to the cause of fibromyalgia is that a trauma or significant stressor turns on an individual’s “fight-or-flight” response. This response, designed to help us function in an emergency situation, usually only lasts a short time, then turns itself off.
But when the stress becomes prolonged, the fight-or-flight response gets stuck in the “on” position and the person’s body remains in a state of high alert. Being in a constant state of high alert puts even more stress on the body. This results in, among other things, a loss of deep, restorative sleep, which in turn causes pain amplification throughout the body.
Regardless of what initially triggers the illness, research has shown that fibromyalgia patients have very real physical abnormalities, including:
- Decreased blood flow to specific areas of the brain, particularly the thalamus region, which may help explain the pain sensitivity and cognitive functioning problems fibromyalgia patients experience.
- High levels of “substance P,” a central nervous system neurotransmitter involved in pain processing.
- Low levels of nerve growth factor.
- Low levels of somatomedin C, a hormone that promotes bone and muscle growth.
- Low levels of several neurochemicals: serotonin, norepinephrine, dopamine and cortisol.
- Low levels of phosphocreatine and adenosine, muscle-cell chemicals.
Since the cause of fibromyalgia remains a mystery, most treatment is aimed at relieving symptoms and improving quality of life. Most patients find that a multi-disciplined approach using a combination of prescription medications, alternative/complementary therapies, gentle exercise and lifestyle adaptations seems to work best.
Unfortunately, finding which medications, therapies, etc. work best for a particular patient is generally a matter of trial and error. What helps one patient may not help another. It’s important that the patient, doctor and other healthcare professionals work together as a team to develop an individualized treatment plan.
(For more information about different treatment options, see”Treatment Modalities.” )
Choosing a Doctor
If you have a primary care physician with whom you’ve established a good rapport, discuss your concerns regarding fibromyalgia. Chances are she has other patients with the same illness, but if not, provide her with information you’ve found helpful. She may or may not remain the gatekeeper in your care, but she should be able to help you find a physician who is familiar with fibromyalgia and able to help you.
In the past, rheumatologists were the specialists who diagnosed and treated fibromyalgia. However, since research is indicating that FM is most likely a central nervous system problem, some rheumatologists are no longer taking FM patients. Check with the rheumatologists in your area to see if they will treat fibromyalgia. Logically, if FM is a central nervous system issue, neurologists would be the specialists that should treat it. Unfortunately, most neurologists are reluctant to add fibromyalgia to their practices. A few, however, have jumped on board. Again, check with the neurologists in your area to see if they will treat FM.
If your doctor is not knowledgeable about FM and is not able to recommend someone, your best resource for finding a doctor who is knowledgeable about fibromyalgia in your area is probably a local support group. (Check ProHealth’s Support Group Listing to find a group near you. ) FM message boards are also a good place to ask if anyone knows of a good doctor in your area. (Visit ProHealth’s FM message board.)
Talking with family and friends may shed some light on your search as well. Hearing of a physician with compassion, one who spends time with patients and listens, goes far when making a decision. Overall, you want to find a physician who is committed to learning as much as she can about the condition so she can provide you with the best possible care.
Do a little research on the doctors you are considering. First, check with your insurance carrier to find out which health care providers are covered by your plan. If there aren’t any suitable doctors in your plan, inquire about out-of-network coverage and charges. Next, check out the doctor’s medical credentials and whether there have been any malpractice suits or disciplinary actions against her. Below are just a few resources to help you begin your research:
- American Medical Association’s Doctor Finder includes physician profiling information such as medical school, training and specialty.
- The American Board of Medical Specialties will tell you if your doctor is board certified and in what specialties. “Board certified” means the doctor has completed two additional years of training and passed a national examination. “Board eligible” means the training, but not the test, has been completed.
- Contact your state’s Board of Medical Examiners to find out about any history of malpractice suits.
- Find out how other patients feel about a doctor you are considering. There are several Web sites, like RateMDs.com where patients rate their doctors. This particular site covers doctors and dentists in the US, Canada, the UK, Australia, New Zealand and India.
As with any chronic illness, navigating your way within the medical world will require you to be your own advocate. This means being proactive about your care, staying informed, and being organized about your needs during each appointment. This is not an easy road, and balancing the medical, insurance and care aspects of your health is going to require clarity and work on your part.
Seldom is fibromyalgia a solitary illness. Most FM patients have one or more comorbid (related) conditions. Which came first is one of those chicken and egg questions.
Why do people with FM usually have so many other disorders? The answer to that may be found in a new paradigm proposed several years ago by Dr. Muhammad Yunus. He suggests that many of these related illnesses could be classified as Central Sensitivity Syndromes. Basically, this means that the central nervous system becomes hypersensitive, which stresses the rest of the body and can eventually lead to any number of different disorders. (See “Are Fibromyalgia and Other Chronic Conditions Associated?” for more information on Central Sensitivity Syndrome.)
Some of the related conditions that fibromyalgia patients may have include:
- Migraine disease
- Restless legs syndrome
- ME/CFS (myalgic encephalomyelitis/chronic fatigue syndrome)
- Irritable bowel syndrome
- Multiple sclerosis
- Temporomandibular joint disorder (TMJ)
- Chiari malformation
- Intermittent cervical cord compression
- Cervical stenosis
- Polymyalgia rheumatica
- Sleep apnea
- Raynaud’s syndrome
- Sjogren’s disease
- Myofascial pain syndrome
- Multiple chemical sensitivity
- Rheumatoid arthritis
- Interstitial Cystitis
- Gulf War Syndrome
Since I found out today this is what I have, I thought I would look into it. I got this information fromt he webite wedmd. Learn more about it. Thanks!
Costochondritis is an inflammation of the junctions where the upper ribs join with the cartilage that holds them to the breastbone or sternum. The condition causes localized chest pain that you can reproduce by pushing on the cartilage in the front of your ribcage. Costochondritis is a relatively harmless condition and usually goes away without treatment. The cause is usually unknown.
- Costochondritis (with unknown cause) is a common cause of chest pain in children and adolescents. It accounts for 10-30% of all chest pain in children. Annually, doctors evaluate about 650,000 cases of chest pain in young people 10-21 years of age. The peak age for the condition is 12-14 years.
- Costochondritis is also considered as a possible diagnosis for adults who have chest pain. Chest pain in adults is considered a potentially serious sign of a heart problem by most doctors until proven otherwise. Chest pain in adults usually leads to a battery of tests to rule out heart disease. If those tests are normal and your physical exam is consistent with costochondritis, your doctor will diagnose costochondritis as the cause of your chest pain. It is important, however, for adults with chest pain to be examined and tested for heart disease before being diagnosed with costochondritis. Often it is difficult to distinguish between the two without further testing. The condition affects females more than males (70% versus 30%). Costochondritis may also occur as the result of an infection or as a complication of surgery on your sternum.
- Tietze syndrome is often referred to as costochondritis, but the two are distinct conditions. You can tell the difference by noting the following:
- Tietze syndrome usually comes on abruptly, with chest pain radiating to your arms or shoulder and lasting several weeks. Tietze syndrome is accompanied by a localized swelling at the painful area (the junction of the ribs and breastbone).
Costochondritis is an inflammatory process but usually has no definite cause. Repeated minor trauma to the chest wall or viral respiratory infections can commonly cause chest pain due to costochondritis. Occasionally, costochondritis as a result of bacterial infections can occur in people who use IV drugs or who have had surgery to their upper chest. After surgery, the cartilage can become more prone to infection, because of reduced blood flow in the region that has been operated on.
Different types of infectious diseases can cause costochondritis.
- Viral: Costochondritis commonly occurs with viral respiratory infections because of the inflammation of costochondral junctions from the viral infection itself, or from straining from coughing.
- Bacterial: Costochondritis may occur after surgery and be caused by bacterial infections.
- Fungal: Fungal infections are rare causes of costochondritis.
Chest pain associated with costochondritis is usually preceded by exercise, minor trauma, or an upper respiratory infection.
- The pain usually will be sharp and located on your front chest wall. It may radiate to your back or abdomen and is more common on your left side.
- The most common sites of pain are your fourth, fifth, and sixth ribs. This pain increases as you move your trunk or take deep breaths. Conversely, it decreases as your movement stops or with quiet breathing.
- The reproducible tenderness you feel when you press on the rib joints (costochondral junctions) is a constant feature of costochondritis. Without this tenderness, a diagnosis of costochondritis is unlikely.
- Tietze syndrome, on the other hand, exhibits swellings at the rib-cartilage junction. Costochondritis has no noticeable swelling. Neither condition involves pus or abscess formation.
- Tietze syndrome usually affects the junctions at the second and third ribs. The swelling may last for several months. The syndrome can develop as a complication of surgery on your sternum months to years after the operation.
- When costochondritis occurs as a result of infection after surgery, you will see redness, swelling, or pus discharge at the site of the surgery.
When to Seek Medical Care
Call the doctor for any of the following symptoms:
- Trouble breathing
- High fever
- Signs of infection such as redness, pus, and increased swelling at the rib joints
- Continuing or worsening pain despite medication
Go to a hospital’s emergency department if you have difficulty breathing or any of the following symptoms occur. These symptoms are generally not associated with costochondritis:
- High fever not responding to fever-reducers such as acetaminophen (Tylenol) or ibuprofen (Advil)
- Signs of infection at the tender spot such as pus, redness, increased pain, and swelling
- Persistent chest pain of any type associated with nausea, sweating, left arm pain, or any generalized chest pain that is not well localized: These symptoms can be signs of a heart attack. If you are not sure what is causing your condition, always go to the emergency department.
Exams and Tests
Costochondritis does not lend itself to diagnosis by tests. Personal history and physical exam are the mainstays of diagnosis. Tests however are sometimes used to rule out other conditions that can have similar symptoms but are more dangerous, such as heart disease.
- The doctor will seek to reproduce tenderness over the affected rib joints, usually over the fourth to sixth ribs in costochondritis, and over the second to third ribs in Tietze syndrome. In costochondritis with unknown causes, there is no significant swelling of costochondral joints.
- There is swelling as well as tenderness of the rib-cartilage junctions in Tietze syndrome. Although some doctors use the terms costochondritis and Tietze syndrome interchangeably, Tietze syndrome has a sudden onset without any preceding respiratory illness or any history of minor trauma. In Tietze syndrome, there is frequently radiation of pain to arms and shoulders as well as pain and tenderness associated with swelling at the spot that hurts.
- Blood work and a chest X-ray are usually not helpful in diagnosing costochondritis. However, after sternum surgery, or for people at risk for heart disease, doctors will be more likely to do tests if you have chest pain and possible costochondritis to be certain you do not have any infection or other serious medical problems.
- They will look for signs of infection such as redness, swelling, pus, and drainage at the site of surgery.
- A more sophisticated imaging study of the chest, a gallium scan, is used to check for infection. It will show increased uptake of the radioactive material gallium in an area of infection.
- In cases of possible infection, the white blood cell count may be elevated.
- Chest X-ray should be obtained if pneumonia is a suspected cause of chest pain.
- ECG and other tests will be done if a heart problem is being considered.
- Costochondritis is a less common cause of chest pain in adults but one that occurs fairly frequently in people who have had cardiac surgery. The diagnosis can only be reached after excluding more serious causes of chest pain that are related to the heart and lungs. The appropriate studies, such as ECG, chest x-rays, blood test for heart damage (cardiac enzymes and troponin levels), and other studies will be done as indicated. Any chest pain in adults is taken seriously and not ignored. If you are concerned, consult with your doctor.
Self-Care at Home
- Treatment involves conservative local care with careful use of nonsteroidal antiinflammatory medications such as ibuprofen (Advil, Motrin) or naproxen (Aleve) as needed.
- Local heat or ice may be helpful in relieving the symptoms.
- Avoid unnecessary exercise or activities that make the symptoms worse. Avoid contact sports until there is improvement in symptoms, and then return to normal activities only as tolerated.
- Costochondritis responds to nonsteroidal antiinflammatory medications such as ibuprofen (Advil or Motrin) and naproxen (Aleve).
- You may be given a local anesthetic and steroid injection in the area that is tender if normal activities become very painful and the pain does not respond to medications.
- Infectious (bacterial or fungal) costochondritis should be treated initially with IV antibiotics. Afterward, antibiotics by mouth or by IV should be continued for another two to three weeks to complete the therapy.
Surgical removal of the sore cartilage may be required if there is no response to medical therapy. Your doctor will refer you to a surgeon for consultation should this option be considered necessary.
You should see a doctor during recovery, and then once a year. Infectious costochondritis requires long-term, close follow-up.
Because inflammatory costochondritis has no definite cause, there is no good way to prevent it.
Noninfectious costochondritis will go away on its own, with or without antiinflammatory treatment. Most people will recover fully.
Infectious costochondritis responds well to IV antibiotics and surgical repair, but recovery may take a long time.
This post is yet another in the long lost of things that have gone wrong for me lately. I am telling this because I am wanting others who are “normal” to understand how a simple thing like a cold can become something even more menacing when you have an autoimmune disease.
It started out innocent enough, just a cold. I don’t know where I got it, but I got it and it wasn’t that bad, at first that is. My husband got it and so did my mother-in-law (who lives with us). This is where the similarities end.
I began to have a fever and chills, sore throat, body aches, ear aches, headaches, swollen lymph nodes in my neck and under my arms, a non-productive cough, tons of sneezing, my face began to look like a malar rash was coming in, and my face was swollen from the sinuses being so full, and a tightness in my chest. I could not eat, felt worse than I normally do, and was basically laying around.
This happened over the weekend so I did not call the doctor, but I tried home remedies that I have used over the years to help me muddle through it. I made honey and lemon tea (I wish I could have gotten some good whiskey but it was not happening), I kept hot or cold packs on my head and face (depending on whether I was hot or cold I used the opposite), I used my neti pot more frequently, I made sure to keep hydrated, and I used my nebulizer for the dry cough.
A couple of days into this and I felt better and thought the worst was over. WRONG! I had a brief reprieve for a day then back into the abyss again. This was on Monday so I broke down and called my rheumy. He ordered me prednisone to help me get on track quicker and prevent the flare I am in from getting worse and to keep me from pleurisy (which I can get easily with these things happening). I then talked to my family doc who ordered me some antibiotics.
It has been two days since them and I think the corner is getting turned. I got out into the cold and snow to go to the store with my hubs. It was hard to do since I am still hurting, but I did it. I am not patting myself on the back, just stating a fact.
On to the next thing… can you believe that there are people out there who actually think I was being a BABY about a mere cold??? That is why I posted the posts about lupus and the flu in an attempt to educate so others will not open their mouths and insert their feet. I mean, come on, if I can live in pain for most days, why should I gripe about a mere cold? If it is overriding the pain I normally feel, then listen up! I am in PAIN and I AM SICK! My lungs get weak quick ( I have asthma) when I get these bugs and it is not something I can buck up and get over!
It really burns me that there are people out there who think I am being a baby. I say, if you have not walked in my shoes, then tread lightly and do not impose your limited medical knowledge on me. Believe me, if I could, I would “get over it”. I would love to walk in your shoes and have the energy to get all I want done. I would love to be able to have a mere cold and not be any sicker. I would love to not lay in a recliner or bed on the days when I am the most sick. I would love to work again!
The truth is that I cannot do a lot of these things anymore and if you need to put me down with your snide remarks and innuendo to make yourself feel better about yourself, then take a long walk off a short pier please and leave me alone. I cherish the real friends I have around me and I do not need anyone who has to be mean to others to elevate their own stature (at least in their mind). It is petty, small minded and just plain ugly to treat others with so much disdain that you belittle their illness and them and then can go off and laugh later thinking you were so clever.
I hate to break it to you but the truth is, you really make a fool of yourself because others can see through you and actually feel pity for you, because you cannot or will not ever understand the feeling of empathy. I feel sorry for you too.
In the meantime, I will deal with each bump along the way and I am so thankful that I am here for today! Little things are big things to me! I am blessed int hat I have dear friends who genuinely love me,and I do not need the pettiness.
I think when I am sick these things hurt me more than when I am feeling better. I apologize for the negativity. Sometimes, I just have to get it out or I will explode.
I hope everyone is safe and warm and dry out there! Thanks for letting me vent!
A friend of mine suffers from this so I wanted to share the information for all to read and learn. Thanks to Renee Proctor, and all the others who deal with this for being brave enough to share this information with others! This information came from www.dystonia-foundation.org.
What is Dystonia?
Dystonia affects people of all ages andbackgrounds.Photo courtesy of Blind Dog Films.
Dystonia is a movement disorder that causes the muscles to contract and spasm involuntarily. The neurological mechanism that makes muscles relax when they are not in use does not function properly. Opposing muscles often contract simultaneously as if they are ‘competing’ for control of a body part. The involuntary muscle contractions force the body into repetitive and often twisting movements as well as awkward, irregular postures. There are approximately 13 forms of dystonia, and dozens of diseases and conditions include dystonia as a major symptom.
Dystonia may affect a single body area or be generalized throughout multiple muscle groups. Dystonia affects men, women, and children of all ages and backgrounds. Estimates suggest that no less than 300,000 people in North America are affected. Dystonia causes varying degrees of disability and pain, from mild to severe. There is presently no cure, but multiple treatment options exist and scientists around the world are actively pursuing research toward new therapies.
Although there are multiple forms of dystonia and the symptoms of these forms may outwardly appear quite different, the element that all forms share is the repetitive, patterned, and often twisting involuntary muscle contractions.
Dystonia is a chronic disorder, but the vast majority of dystonias do not impact cognition, intelligence, or shorten a person’s life span. The main exception to this is dystonia that occurs as symptom of another disease or condition that can cause such complications.
I know several people who have this and so it seemed only natural to include an overview here. The name can be deceiving because it is not only associated with the legs. Please read so you can understand and learn more. It was an eye opening article for me. This information is from www.rls.org website. While this article is short, you can check into it more at the website and many others.
Restless Leg Syndrome
Restless legs syndrome (RLS) is a neurological condition that is characterized by the irresistible urge to move the legs. While the name may sound funny, it is a very real disorder. In order for you to be officially diagnosed with RLS, you must meet the criteria described in the four bullets below:
- You have a strong urge to move your legs which you may not be able to resist. The need to move is often accompanied by uncomfortable sensations. Some words used to describe these sensations include: creeping, itching, pulling, creepy-crawly, tugging, or gnawing.
- Your RLS symptoms start or become worse when you are resting. The longer you are resting, the greater the chance the symptoms will occur and the more severe they are likely to be.
- Your RLS symptoms get better when you move your legs. The relief can be complete or only partial but generally starts very soon after starting an activity. Relief persists as long as the motor activity continues.
- Your RLS symptoms are worse in the evening especially when you are lying down. Activities that bother you at night do not bother you during the day.
RLS can also cause difficulty in falling or staying asleep which can be one of the chief complaints of the syndrome. A substantial number of people who have RLS also have periodic limb movements of sleep (PLMS). These are jerks that occur every 20 to 30 seconds on and off throughout the night. This can cause partial awakenings that disrupt sleep. Sleep deprivation can seriously impact your work, relationships, and health.
MOST COMMON SYMPTOMS AND SIGNS
OF BEHCET’S DISEASE
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.
Aneurysms (outpouchings of blood vessel walls, caused by inflammation) of arteries in the lungs, rupture of which may lead to massive lung hemorrhage.
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:
recurrent oral ulcerations (apthous or herpetiform) at least three times in one year
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.