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