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Systemic lupus erythematosus (SLE) is a disease with a fluctuating course. Nervous system manifestations can occur at any time, even when no non–nervous system SLE activity is detected.1 To increase consistency in the classification of patients and standardize clinical reporting requirements in neuropsychiatric SLE (NPSLE) research, the American College of Rheumatology (ACR) established case definitions for 19 different neurologic, psychiatric, and cognitive NPSLE syndromes.2
Two articles in this issue of Neurology discuss important aspects of NPSLE. Using ACR case definitions, Afeltra et al. examined the relationship between NPSLE syndromes and antiphospholipid antibodies (aPL).3 Chau and Mok assessed factors predictive of corticosteroid-induced psychosis, a difficult clinical problem.4
Estimates of the prevalence of NPSLE have ranged from 14% to over 80%.5-7⇓⇓ All but the most recent studies are based on research conducted before the introduction of the ACR case definitions. Syndromes most commonly seen include cerebrovascular disease (prevalence 2 to 24%), total spectrum of headache (21 to 61%), total spectrum of mood disturbances (27 to 74%), psychosis (0 to 5%), and total range of cognitive disorders (52 to 80%). The majority of patients have more than …
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