FYI *Re: Lupus Cerebritis and Cerebral Vasculitis*
The suffix “itis” means inflamed, so one would expect to see inflammatory changes in the brains of SLE patients.
However, various studies have demonstrated minimal inflammation, particularly of the blood vessels.
Therefore, the terms above should be considered misnomers and it is preferable to use the terms either/or NP-SLE, CNS-SLE.

SYMPTOMS or Manifestations of CNS-SLE

In addition to headache, NP-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.
Characteristic abnormalities are frequently found on brain MRI, lumbar puncture and electroencephalogram.

The diagnosis is not always straightforward.

Although there are no “definitive” tests for CNS-lupus, there is a type of brain scan called a “SPECT brain scan” that may be positive even when an MRI brain scan is normal.
There is also a blood test called an anti-ribosomal-P-antibody that, when it is positive, may be helpful in establishing the diagnosis.

INDICATORS for Diagnosis of CNS-SLE

NP-SLE is extremely variable.
The revised criteria for the diagnosis of NP-SLE lists only coma and seizures, however, the clinical indications of the disease can vary from profound and life threatening neurological disturbances to ongoing mild complaints such as headaches or behavioral disturbances.

Further confounding the ability to diagnose NP-SLE is the fact that a patient may develop a psychiatric illness as a consequence of having lupus without having NP-SLE.
Eg: ‘reactive depression‘ or “Why me?” and/or the patient may develop a psychiatric illness entirely separate from the lupus diagnosis entirely.

Also, certain therapies may produce psychiatric disturbances.
Steroid therapy, particularly in high doses, can produce psychotic behaviors.
As well, the fact that many patients with systemic lupus present with cognitive function difficulties, makes it a challenge for the physician to differentiate between what is related to active systemic LUPUS, what is related to corticosteroid treatment, and what may be related to depression or neuropsychiatric lupus.

As the therapy used to treat patients with SLE can cause psychiatric problems, it makes it very difficult for the rheumatologist to decide if the patient is suffering from primarily NP-SLE, reactive depression or the side effects of steroid therapy.

It is usually necessary to rule out other conditions that may mimic central nervous system manifestations of systemic lupus erythematosus, including infection and toxic metabolic states.

CNS-SLE DIAGNOSIS

The rheumatologist has a battery of diagnostic tests and procedures which can aid him in making the diagnosis of NP-SLE.
These include studies of spinal fluid, Brain Scan, Electroencephalography (EEG), Cat Scan and psychometric testing.
But at present, there is no single test or group of procedures that can unerringly diagnose the illness in all cases.
The tests can merely increase the index of suspicion that NP-SLE is present.

To put this in the proper perspective, a patient who has multi-organ involvement and signs of severe NP-SLE, such as seizures, strokes, etc., can easily be given the diagnosis of NP-SLE.
However, these patients represent the exception to the rule.
The typical patient shows joint pain, skin rash, a positive ANA and mild symptoms of NP-SLE such as headaches and severe anxiety.

For such a patient, the rheumatologist is confronted with the difficult decision of determining if the headaches or anxiety are truly caused by NP-SLE.
Moreover, for certain patients, their only indications of SLE may be NP-SLE, further adding to the diagnostic quandry.
Multiple investigators are attempting to find better tests to diagnose NP-SLE, such as the test for antinerve cell antibodies.

TREATMENT of CNS-SLE

Since the indications of NP-SLE can vary from very severe to mild symptoms, should all patients with NP-SLE be treated with steroids, irrespective of the intensity of the disease activity?
This is a controversial subject among rheumatologists.

Patients with central nervous system manifestations of lupus erythematosus who present with organic brain syndrome or coma can be treated with intravenous methylprednisolone pulse therapy.
Patients with severe or resistant symptoms may also require treatment with intravenous cyclophosphamide and/or plasmapheresis.

Conclusively, individuals with major manifestations of NP-SLE require aggressive therapy with high dose steroids and perhaps immunosuppressive drugs, plus other appropriate treatment such as anti-seizure medication.

But what should be done for patients with minor manifestations of neurologic and psychiatric NP-SLE?
At present, the treatment of symptoms is most judicious, such as the control of headaches and migraines with appropriate medications.

CONCLUSIONS

The study of NP-SLE is still in its infancy, but certain strides are being made.
For example, it appears that at least one subset of patients with NP-SLE has been identified.
In these patients, NP-SLE is indicated by strokes, apparently because an autoantibody predisposes the brain to develop blood clots leading to strokes.
An enormous amount of investigative effort is required to further understand the mechanisms relating to NP-SLE.
Hopefully a better understanding of the involved mechanisms will lead to the development of more rational therapy for the many manifestations of CNS-SLE.