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OBJECTIVE: To determine the diagnostic and therapeutic approach used in clinical practice for the management of systemic lupus erythematosus (SLE) patients with primary SLE-mediated neuropsychiatric (NP) manifestations. METHODS: A questionnaire was drawn up to assess how clinicians manage various clinical manifestations of SLE. A portion of this questionnaire was designed to assess how clinicians diagnose and treat primary NP-SLE. Most of the questions in the NP-SLE section consisted of lists of different clinical manifestations and laboratory or radiological studies that participants were asked to rate on a scale of importance [from 1 (extremely important) to 5 (not important)] to the diagnosis of primary NP-SLE. The questionnaire also assessed how different NP manifestations are treated in clinical practice. The relative importance of each clinical manifestation was determined through its mean score, and the agreement among participants on each issue was determined using the coefficient of variation (CV). Fifty-nine lupus centers participated in the NP-SLE portion of the survey. RESULTS: The clinical manifestations which were considered to be of extreme or major importance for the diagnosis of primary NP-SLE were seizures, psychosis, transverse myelitis, stroke, transient ischemic attack (TIA) and aseptic meningitis. Among the radiological and laboratory studies, only brain magnetic resonance imaging (MRI) and antiphospholipid antibodies (aPL) achieved "extremely important" mean scores (between 1 and 2). aPL testing was used routinely in the majority of patients (mean 96.8%; CV = 0.1), while brain MRI was used less frequently (mean 56.5%; CV = 0.61). Only brain MRI and cerebral angiography were considered to be helpful in differentiating cerebral vasculopathy from multi-infarct (mean score = 1.6 and 1.9, respectively), whereas a prompt response to treatment with increased doses of steroids was considered helpful in differentiating SLE-related psychosis from steroid-induced psychosis (mean score = 1.58). The results of aPL testing, coagulation tests for the lupus anticoagulant, an brain MRI were considered to be of extreme or major importance in decisions involving treatment with anticoagulant or anti-platelet therapy. Symptomatic therapies, such as heparin, or anti-convulsant, anti-platelet, oral anticoagulant, and antipsychotic therapy were the most widely used. Corticosteroids were the most frequently used immunosuppressive therapy. The administration of other immunosuppressive agents as specific treatment for NP-SLE was uncommon. CONCLUSIONS: Our survey found that in clinical practice, the NP manifestations currently considered to be diagnostic of primary SLE-mediated CNS involvement are not limited to those included in the American Rheumatism Association (ARA) criteria, e.g. seizures and psychosis. Antiphospholipid antibodies appeared to be the laboratory parameter most frequently relied upon in the diagnosis of NP-SLE, and in decisions regarding treatment. Apart from that, only brain MRI and, in selected cases, cerebral angiography seemed to be of real help in diagnosis. The lack of consensus regarding the treatment of primary NP-SLE manifestations most probably reflects both the complex nature of neurological illness in SLE patients and the lack of clear diagnostic criteria.