FRI0392 Efficacy of Rituximab in Systemic Manifestations of Primary Sjogren's Syndrome
Authors: O. Logvinenko, V. Vasilyev, E. Alexandrova, E. Rodionova, T. Safonova, E. Nasonov
Journal: Annals of the Rheumatic Diseases (2014)

<h3>Background</h3> There is lack sufficient data on efficacy of Rituximab (RTX) in primary Sjogren9s syndrome (pSS) with systemic manifestations. <h3>Objectives</h3> To evaluate the efficacy of RTX in patients with systemic manifestations of primary pSS. <h3>Methods</h3> 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 (&lt;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 didn9t 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 &gt;50% in baseline parameters. All patients fulfilled the ACR 2012 criteria for pSS. <h3>Results</h3> 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&lt;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 didn9t change (0.64 (0.32-0.73) before and 0.65 (0.62-1.82) ng/ml after treatment). RTX treatment didn9t increase stimulated whole saliva secretion (median 0 ml). The Schirmer9s 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) didn9t improve. <h3>Conclusions</h3> RTX is good prescription for treatment of pSS patients with systemic manifestation. <h3>References</h3> Gottenberg J, et al. Efficacy of rituximab in systemic manifestations of primary Sjogren9s syndrome: results in 78 patients of the AutoImmune and Rituximab registry. Ann Rheum Dis 2012;0:1-6.doi:10.1136/annrheumdis-2012-202293 <h3>Acknowledgements</h3> The author would like to thank the Nasonova Research Institute of Rheumatology <h3>Disclosure of Interest</h3> None declared <h3>DOI</h3> 10.1136/annrheumdis-2014-eular.5201