Neuropsychiatric systemic lupus erythematosus

Definitions and diagnostic criteria are established for 19 CNS and PNS syndromes cases observed in systemic lupus erythematosus (SLE) patients, which collectively are referred to as neuropsychiatric systemic lupus erythematosus (NPSLE) syndromes. An important consideration in the diagnostic approach to a patient with possible NPSLE manifestations is whether the particular clinical syndrome is due to SLE-mediated organ dysfunction, a secondary phenomenon related to infection, medication side-effects, or metabolic abnormalities (e.g., uremia), or is due to an unrelated condition. For each NPSLE syndrome, the ACR case definitions give a clear guidance regarding the differential diagnosis and evaluation that is required to make a diagnosis of a primary NPSLE syndrome. The presence of discoid and articular manifestations is thought to associate with a more benign course and less likelihood of NPSLE [20], while their absence at onset or during flares seems to bestow a higher risk of CNS involvement, primarily cerebrovascular disease and seizures, and also psychosis, acute confusional state, and isolated cognitive dysfunction. Other associations with high likelihood of NPSLE are low serum levels of C3, and especially C4, high-titer aCL (IgG) antibodies, and cutaneous vasculitic lesions. There is no single diagnostic test sensitive and specific for NPSLE. The assessment of individual patients is based on clinical neurological and rheumatologic evaluation, immunoserologic testing, brain imaging, and psychiatric and neuropsychologic assessment.