• C Vitali, A Doria, A Tincani, P Fabbri, G Balestrieri, M Galeazzi, P L Meroni, P Migliorini, R Neri, A Tavoni, and S Bombardieri.
• Rheumatology Unit, University of Pisa, Italy.
• Clin Exp Rheumatol. 1996 Nov 1; 14 Suppl 16: S17-22.

ObjectiveThe diagnosis and treatment of the mucocutaneous (MC), neuropsychiatric (NP), and renal (RN) manifestations of systemic lupus erythematosus (SLE) remain unsolved issues. To shed light on these issues, a questionnaire was prepared and sent to 153 lupus centres around the world, in order to determine the level of agreement between experts in their approach to these complex aspects of the disease.MethodsThe first section of the questionnaire was designed to collect information on the characteristics of the responding lupus centres. The second section was dedicated to MC manifestations, with questions focusing on: (i) the frequency of MC manifestations as a whole and of the single clinical MC entities; (ii) clinical features, outcome and therapy of subacute cutaneous lupus erythematosus (SCLE); (iii) the utility of the lupus band test (LBT); and (iv) the use of various therapeutic protocols to treat MC manifestations.ResultsSixty-one questionnaires from 19 countries were analysed. Out of these, 37 were completed by Departments of Rheumatology, 21 by Departments of Internal Medicine or Clinical Immunology, and 3 by Departments of Nephrology. About 66% of these centres stated that they were currently following more than 100 lupus cases, 95% had an in-patient ward and 82% had their own laboratory. The American College of Rheumatology classification criteria and various scales for disease activity assessment were regularly used by 87% and 57% of centres respectively. The overall prevalence of MC manifestations was judged to be over 30% by 82% of the respondents (Rs), and over 60% by 36% of the Rs. Among the different MC manifestations, malar rash was reported to be the most frequent (40%), followed by alopecia (24.1%) and oral ulcers (18.6%). In reporting the prevalence of each MC manifestation, the Rs showed a low level of agreement, the coefficient of variation (CV) being > 0.75 for all of the manifestations listed with the exception of malar rash (CV = 0.54). Poor agreement among centers was also found for the reported association of various MC manifestations with SCLE (15 different answers), and on the prognostic factors for SCLE (17 different answers). There was agreement on the best procedure (up to 70% of the Rs preferred a non-UV exposed skin area) and on the utility of the LBT (83% using it only for diagnostic purpose). Hydroxychloroquine was the most popular therapeutic protocol, being used by 85% of the Rs for a wide variety of MC manifestations. Among other therapies, azathioprine was used by 59%, dapsone by 41%, and thalidomide by 35% of the Rs, all to treat a wide spectrum of MC manifestations. Pulse steroid, cyclosporin A and pulse cyclophosphamide were less commonly employed (by 27%, 22% and 13% of the Rs, respectively), and were reserved for the most severe MC manifestations, particularly vasculitis.ConclusionThe present survey indicates that, although most of the participating centres had extensive experience in the management of SLE, their approach to the MC manifestations was not homogeneous, and collaborative studies are clearly needed, particularly to optimise the therapeutic protocols.

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