**3.4 Central nervous system**

Roughly 32% of lupus patients develop lupus that attacks the central nervous system [29]. Lupus involving the central nervous system is both a confusing and interesting aspect of the disease [36, 37]. Virtually any central nervous system or peripheral nervous system problem including, but not limited to, neuropathy, mononeuritis multiplex, seizures, blindness, loss of hearing, cranial nerve palsy, encephalopathy, psychosis and movement disorders, are not uncommon in the lupus population, and may frequently present as an initial feature of the disease.

To reemphasize, all symptomatology that has been mentioned in this chapter may be an initial feature of lupus; however, the lack of swift rheumatology involvement often ultimately leads to a delay in diagnosis, which is always detrimental to the patient. Therefore, it is important to perform a comprehensive evaluation, including biopsy, angiogram, or other internal organ imaging, as well as complete serologic testing. Additionally, most patients are not willing to take medication for extended periods of time, unless it can be proven to them by their physician that the medication will indeed benefit them by alleviating the symptoms they are experiencing. This will assist in a more accurate diagnosis of lupus versus another disease process. As in every case involving a possible autoimmune process, emphasis should be placed on the importance of swift initiation of workup, as this will facilitate the timely establishment of proper treatment.

If a patient is acutely ill with psychosis, they will typically be treated in a hospital setting, being initially seen by neurology and psychiatry, as other specialists. Unfortunately, this occurs before a rheumatologist is consulted [38]. An immediate MRI of the brain and lumbar puncture should be ordered, along with autoantibodies and cerebrospinal fluid, to assess the ribosomal P antibody, GAD65 antibody and NMO**.** With these proper evaluations, the likelihood of a CNS lupus diagnosis may be determined.

It is quite typical in that lupus patients, including those with renal and central nervous system involvement, in general, do quite well with medical compliance. Published death rates, transplant rates, and dialysis rates for lupus nephritis are decidedly dependent upon the population type that is investigated. A well-educated compliant group of patients has a very low incidence of end stage renal disease while the noncompliant group almost certainly ultimately develop end stage renal disease [39].
