INTERNATIONAL SURVEY ON THE MANAGEMENT OF PATIENTS WITH SLE .2. THE RESULTS OF A QUESTIONNAIRE REGARDING NEUROPSYCHIATRIC MANIFESTATIONS

Objective. To determine the diagnostic and therapeutic approach used i n clinical practice for the management of systemic lupus erythematosus (SLE) patients with primary SLE-mediated neuropsychiatric (NP) manife stations. Methods. A questionnaire was drawn up to assess how clinicia ns manage various clinical manifestations of SLE. A portion of this qu estionnaire was designed to assess how clinicians diagnose and treat p rimary NP-SLE. Most of the questions in the NP-SLE section consisted o f lists of different clinical manifestations and laboratory or radiolo gical studies that participants were asked to rate on a scale of impor tance [from 1 (extremely important) to 5 (not important)] to the diagn osis of primary NP-SLE. The questionnaire also assessed how different NP manifestations are treated in clinical practice. The relative impor tance of each clinical manifestation was determined through its mean. score, and the agreement among participants on each issue was determin ed using the coefficient of variation (CV). Fifty-nine lupus centers p articipated in the NP-SLE portion of the survey. Results. The clinical manifestations which were considered to be of extreme or major import ance for the diagnosis of primary NP-SLE were seizures, psychosis, tra nsverse 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 t esting 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 = 0.6 1). Only brain MRI and cerebra I angiography were considered to be hel pful in differentiating cerebral vasculopathy from multiinfarct diseas e (mean score = 1.6 and 1.9, respectively), whereas a prompt response to treatment with increased doses of steroids was considered helpful i n differentiating SLE-related psychosis from steroid-induced psychosis (mean score = 1.58). The results of aPL testing, coagulation tests fo r the lupus anticoagulant, and brain MRI were considered to be of extr eme or major importance in decisions involving treatment with anticoag ulant or anti-platelet therapy. Symptomatic therapies, such as heparin , or anti-convulsant, anti-platelet, oral anticoagulant, and antipsych otic therapy were the mast widely used. Corticosteroids were the most frequently used immunosuppressive therapy. The administration of other immunosuppressive agents as specific treatment for NP-SLE was uncommo n. Conclusions. Our survey found that in clinical practice, the NP man ifestations currently considered to be diagnostic of primary SLE-media ted CNS involvement are not limited to those included in the American Rheumatism Association (ARA) criteria e.g. seizures and psychosis. Ant iphospholipid antibodies appeared to be the laboratory parameter most frequently relied upon in the diagnosis of NP-SLE, and in decisions re garding treatment. Apart from that, only brain MRT and, in selected ca ses cerebral angiography seemed to be of real help in diagnosis. The l ack of consensus regarding the treatment of primary NP-SLE manifestati ons most probably reflects both the complex nature of neurological ill ness in SLE patients and the lack of clear diagnostic criteria.