Treatment of lupus psychosis with oral cyclophosphamide followed by azathioprine maintenance: an open-label study

Abstract

Neuropsychiatric disease in systemic lupus erythematosus can have diverse manifestations, ranging from subtle cognitive dysfunction to overt life-threatening diseases such as stroke and status epilepticus (1). The prevalence of neuropsychiatric manifestations varies widely, from 9% to 80% in various series, depending on the definition, method of ascertainment, pattern of referral, and patient ethnicity (1– 4). Neuropsychiatric lupus carries substantial morbidity and is a marker of decreased survival (2,5,6). Aggressive treatment is warranted for severe manifestations, such as psychosis, acute confusional state, transverse myelopathy, optic neuritis, demyelinating disease, and peripheral neuropathy. Because of the lack of controlled trials, treatment is often based on clinical experience. Successful treatment of severe lupus nephritis with corticosteroids and cyclophosphamide (7–12) has led to the use of these agents in other serious manifestations of lupus, and favorable responses have been reported with the use of steroids and intravenous pulse cyclophosphamide for neuropsychiatric systemic lupus erythematosus (2,13–18). However, these studies included patients with various neuropsychiatric syndromes, and outcomes such as relapses of neuropsychiatric disease were not reported. In the current study, we focused on lupus psychosis and report the outcome of patients treated with steroids and oral cyclophosphamide initially, followed by azathioprine maintenance.