FRI0253 A 18 Month Observation in Patients with Progressive Systemic Sclerosis Treated with An Intensified B-Lymphocyte Depletion -Clinical and Immunological Response

Background B cells (BC) play a critical role in progressive systemic sclerosis (SS). BC depletion therapy is an attractive option. Objectives to evaluate the efficacy of an intensified B cell depletion therapy in patients with progressive SS Methods Eighteen patients with SS [2 males, mean age 58.33 yrs (46–82)] with severe multiorgan involvement including skin [16], lung, with pulmonary fibrosis (17 patients) and pulmonary hypertension (5), esophagous [9], with polyarthralgias [2] have been prospectively treated with an intensified B cell depletion therapy (IBCDT) protocol due to their resistance or intolerance to previous therapy (4 cases) or as a front line treatment in 14 patients with unsatisfactory therapeutic compliance. IBCDT included: Rituximab (RTX) 375mg/sm on days 1, 8, 15, 22, and 2 more doses after 1 and 2months, associated with 2 IV administrations of 10mg/kg of cyclophosphamide and 3 methylprednisolone pulses (15mg/kg) followed by oral prednisone (0.8mg/kg/day, rapidly tapered to 5mg/day by the end of the 3rd month after RTX). No further immunosuppressive maintenance therapy has been given. Results Patients had been followed-up for a mean of 21,67 ± ds 11,05 (06–43) months. Significant decreases (p Conclusions A promising role of RTX in an intensified protocol of induction therapy can be envisaged in patients for whom avoiding prolonged immunosuppressive therapy particularly appealing. Our data confirm the opportunity to consider the regimens of BL depletion in the treatment of the most severe or refractory forms of SS. Disclosure of Interest None declared