Efficacy of Anti-CD Monoclonal Antibody in SLE-Related Sneddonandrsquo;s Syndrome with Anti phospholipid Antibodies and Interstitial Lung Disease: A Case Report

Abstract

Sneddon’s Syndrome (SS) is a rare condition characterized by a non-inflammatory thrombotic vasculopathy, which involves small and medium vassels. Cerebrovascular disease and livedo racemosa are the main clinical manifestations of this syndrome. The incidence of SS has been estimated of 4 cases per 1 million/year in general population with a high prevalence in young women between 20-40 years of age. Three forms of the syndrome have been described: idiopathic, without a clear causative factor, related to primary Antiphospholipid Syndrome and related to Systemic Lupus Erythematosus (SLE) with or without anti-phospholipid antibodies. To date, there are very few indications for diagnosis and treatment. Skin biopsy and brain MRI are considered fundamental in the diagnostic process; however histologic samples could be negative or non-specific. Current treatment is based on oral anticoagulation and, in autoimmune-related SS, on immunosuppressant drugs, such as corticosteroids, cyclophosphamide, azathioprine, with unclear results. No data about the use of anti-CD20 monoclonal antibodies (Rituximab) in autoimmune-related SS are available so far. We report here, for the first time, the case of a 40 years old woman with SLErelated SS with anti-phospholipid antibodies, successfully treated with Rituximab.