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When dealing with neuropsychiatric Systemic Lupus Erythematosus (NPSLE) there are still many controversial topics. In 1999 the American College of Rheumatology gave classification criteria for 19 clinical syndromes. However major problems are still related to low specificity of some of them such as headache, cognitive impairment or mood disorders. Even though a frequency of CNS involvement from 14 to 75% has been described, depending on both the population studied and the methodology of assessment, a lower frequency ranging from 21 to 28 % derived by larger case series seems more realistic. The introduction of the concept of “borderline cases”, proposed by Italian Study Group for NP-SLE, is based both on clinical and instrumental evaluation and could represent a useful tool when dealing with conditions which do not fulfil ACR classification. Also the relationship between SLE activity and NP involvement is a debated issue. Concerning pathogenesis, it seems reasonable to consider multifactorial mechanisms related to antibody-mediated damage, antiphospholipid pro-thrombotic effect, non-inflammatory vasculopathy and cytokines mediated cytotoxycity. However, direct and unequivocal evidence for the implication of any of the above-mentioned mechanisms is still lacking. Although a wide range of neuroimaging tools have been used to evaluate CNS involvement, no single technique has proven to be definitive and, when dealing with a patient with suspected NPSLE, it is important to combine different diagnostic techniques. Due to the lack of effective imaging along with limitation in knowledge of underlying pathogenetic mechanisms and paucity of histopathologic findings, therapeutic approach in NPSLE remains a difficult issue and is currently based on personal experience. Italian Study Group for NP-SLE proposes the creation of a national registry on NPSLE to validate ACR classification criteria. Furthermore, the possibility to collect large series and stratifying them for each of the included neuro-psychiatric syndromes seems a good strategy for planning multicentric controlled therapeutic trials in the near future.
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