TY - JOUR T1 - Efficacy of rituximab in systemic manifestations of primary Sjögren's syndrome: results in 78 patients of the AutoImmune and Rituximab registry JF - Annals of the Rheumatic Diseases JO - Ann Rheum Dis SP - 1026 LP - 1031 DO - 10.1136/annrheumdis-2012-202293 VL - 72 IS - 6 AU - Jacques-Eric Gottenberg AU - Gael Cinquetti AU - Claire Larroche AU - Bernard Combe AU - Eric Hachulla AU - Olivier Meyer AU - Edouard Pertuiset AU - Guy Kaplanski AU - Laurent Chiche AU - Jean-Marie Berthelot AU - Bruno Gombert AU - Philippe Goupille AU - Christian Marcelli AU - Séverine Feuillet AU - Jean Leone AU - Jean Sibilia AU - Charles Zarnitsky AU - Philippe Carli AU - Stephanie Rist AU - Philippe Gaudin AU - Carine Salliot AU - Muriel Piperno AU - Adeline Deplas AU - Maxime Breban AU - Thierry Lequerre AU - Pascal Richette AU - Charles Ghiringhelli AU - Mohamed Hamidou AU - Philippe Ravaud AU - Xavier Mariette Y1 - 2013/06/01 UR - http://ard.bmj.com/content/72/6/1026.abstract N2 - Objectives To evaluate the efficacy and safety of rituximab in patients with primary Sjögren's syndrome (pSS). Methods The AutoImmune and Rituximab registry has included 86 patients with pSS treated with rituximab, prospectivey followed up every 6 months for 5 years. Results Seventy-eight patients with pSS (11 men, 67 women), who already had at least one follow-up visit, were analysed. Median age was 59.8 years (29–83), median duration of disease was 11.9 years (3–32). Indications for treatment were systemic involvement for 74 patients and only severe glandular involvement in four patients. The median European Sjögren's Syndrome disease activity index (ESSDAI) was 11 (2–31). 17 patients were concomitantly treated with another immunosuppressant agent. Median follow-up was 34.9 months (6–81.4) (226 patient-years). Overall efficacy according to the treating physician was observed in 47 patients (60%) after the first cycle of rituximab. Median ESSDAI decreased from 11 (2–31) to 7.5 (0–26) (p<0.0001). Median dosage of corticosteroid decreased from 17.6 mg/day (3–60) to 10.8 mg/day (p=0.1). Forty-one patients were retreated with rituximab. Four infusion reactions and one delayed serum sickness-like disease resulted in rituximab discontinuation. Three serious infections (1.3/100 patient-years) and two cancer-related deaths occurred. Conclusions In common practice, the use of rituximab in pSS is mostly restricted to patients with systemic involvement. This prospective study shows good efficacy and tolerance of rituximab in patients with pSS and systemic involvement. ER -