Objectives: To evaluate the efficacy and safety of rituximab (RTX) in patients with primary Sjogren's syndrome (pSS).

Methods: The AutoImmune and Rituximab registry (AIR), an independent registry of the French Society of Rheumatology, includes 3662 off-trial patients with refractory autoimmune diseases treated with RTX and prospectivey followed up for 5 years. Efficacy was defined according to the opinion of the physician. Each treating physician was asked to validate the data of his patient(s). ESSDAI scores were retrospectively scored.

Results: In the AIR registry 86 patients (pts) with pSS were treated with RTX. The data have been validated by clinicians for 79 pts. 78 pts who had at least one follow-up visit (median follow-up of 34.9 months [6-81.4], 226 patient/years) were analyzed. Median age (11 males, 67 females) was 59.8 years [29-83], the median duration of pSS was 11.9 years [3-32]. Indications of the treatment were: systemic involvement for 74 pts (articular (n=27), peripheral (PNS)/central nervous system (CNS) (n=12/6), pulmonary (n=9), renal (n=6), muscular (n=3), haematological (n=2) involvements, vasculitis (n=8, including 5 cryoglobulinemias), autoimmune pancreatitis (=1), and only glandular involvement in 4 pts. Median ESSDAI assessed in 78 pts was 11 [2-31]. 67 pts were initially treated by 2 infusions of 1g, 11 pts by 4 infusions of 375mg/m². 17 pts were concomitantly treated with: methotrexate (7 pts), mycophenolate mofetil (1 pt), hydroxychloroquine (7pts), azathioprin (1 pt), cyclophosphamide (1 pt). 29 pts were also treated with corticosteroids (median dosage 18 mg [3-60]). Efficacy: Overall efficacy according to the clinician was observed in 47 pts (60%) after the 1st cycle of RTX. Efficacy was observed in 45 pts (61%) with systemic involvement: articular (63% of pts), PNS (50%), CNS (33%), pulmonary (78%), renal (83%), muscular (0%), haematological (100%) involvement, autoimmune pancreatitis (100%), and vasculitis (62%). No data was available regarding the evolution of dryness. Efficacy was observed in 2 pts (50%) with only glandular involvement (2 parotid swellings). Median ESSDAI after RTX decreased from 11 [2-31] to 7.5 [0-26] (p<0.0001). Mean dosage of corticosteroid after RTX decreased from 18 mg/day [3-60] to 11mg/day (p=0.1). Tolerance: 4 serious immediate infusion reactions and 1 delayed serum-sickness like disease occurred. 3 serious infections occurred (1.3/100 patient/years) and 2 cancers (0.9/100 patient/years). The 2 pts with cancer died. RTX was discontinued in 41 pts (inefficacy: 35, serious adverse events:6). The median number of RTX cycles was 2.3 [1-12]. Median duration between 1st and 2nd cycles was 11 months (5.4-29.4).

Conclusions: This study shows good efficacy and short term safety of RTX for systemic features. This study demonstrates that in real life, clinicians mostly use RTX off-label in pSS patients with systemic involvement. This large prospective study suggests the efficacy of RTX in systemic complications of pSS with a corticosteroid sparing effect. These results have to be confirmed by controlled studies.

Disclosure of Interest: None Declared