Background There is lack sufficient data on efficacy of Rituximab (RTX) in primary Sjogren's syndrome (pSS) with systemic manifestations.
Objectives To evaluate the efficacy of RTX in patients with systemic manifestations of primary pSS.
Methods Twenty-four patients with systemic complications of pSS were treated with RTX. The main clinical manifestations of pSS were cryoglobulinemic purpura and skin ulcers (22/24 (91.7%)), glomerulonephritis (11/23 (48%)), sensorimotor axonal polyneuropathy (13/24 (54.2%)). 21 patients (87.5%) had cryoglobulins (type II, n=18, and type III, n=3). Mean age and median disease duration were 57.6±11.3 years and 10.7 (interquartile range 7-22) years, respectively. Mean dose of RTX was 2.0±0.3 g. RTX as monotherapy was given in 9 patients and in 14 cases RTX was combined with cyclophosphamide (median cumulative dose 4 g (2-4)). BAFF levels were measured in 9 cases by ELISA (<0.8 ng/ml is normal BAFF value). The response to RTX treatment was evaluated at 3 and 6 month after the last infusion. A clinical complete response was defined by the disappearance in all baseline clinical signs. Irreversible damage like sustained and stable neuropathy didn't exclude complete response. A partial response was defined by an improvement of at least half of the baseline clinical signs. A complete immunologic response was defined by the absence of serum cryoglobulins, monoclonal Ig and normalization of the C4 complement fraction level, and partial immunologic response by disappearance and/or normalization >50% in baseline parameters. All patients fulfilled the ACR 2012 criteria for pSS.
Results At month 3 after RTX therapy, a clinical complete and partial response was observed in 71.4% (15/21) and 19% (4/21) cases, respectively. A complete/partial immunologic response was presented in 52.6% (10/19)/26.3% (5/19) patients. At month 6 clinical and immunologic relapse was noted in 25% (5/20) and 35.3% (6/17) cases, respectively. There was decrease median ESSDAI score from 8 (7-10) at baseline to 3 (2-4) at month 3 and to 3 (2-5) at month 6 (p<0.001). At the baseline in 9 patients median serum BAFF concentration was 1.71 (0.66-2.73) ng/ml. In 5 patients who had high baseline levels of BAFF median BAFF concentration decreased from 2.73 (2.66-2.74) to 0.76 (0.68-2.58) ng/ml after RTX therapy. In 4 patients with low levels of BAFF before RTX treatment BAFF didn't change (0.64 (0.32-0.73) before and 0.65 (0.62-1.82) ng/ml after treatment). RTX treatment didn't increase stimulated whole saliva secretion (median 0 ml). The Schirmer's test (median 4 (2-12) before and 5 (2-10) mm after RTX) and tear break-up time (median 7 (6-10) before and 5 (5-9) sec after RTX) didn't improve.
Conclusions RTX is good prescription for treatment of pSS patients with systemic manifestation.
Gottenberg J, et al. Efficacy of rituximab in systemic manifestations of primary Sjogren's syndrome: results in 78 patients of the AutoImmune and Rituximab registry. Ann Rheum Dis 2012;0:1-6.doi:10.1136/annrheumdis-2012-202293
Acknowledgements The author would like to thank the Nasonova Research Institute of Rheumatology
Disclosure of Interest None declared