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Efficacy and safety of rituximab treatment in early primary Sjögren's syndrome: a prospective, multi-center, follow-up study

Francesco Carubbi1*, Paola Cipriani1, Alessandra Marrelli1, Paola Di Benedetto1, Piero Ruscitti1, Onorina Berardicurti1, Ilenia Pantano1, Vasiliki Liakouli1, Saverio Alvaro1, Alessia Alunno2, Antonio Manzo3, Francesco Ciccia4, Roberto Gerli2, Giovanni Triolo4 and Roberto Giacomelli1

Author Affiliations

1 Department of Clinical Science and Biotechnology, Rheumatology Unit, University of L’Aquila, 67100 L’Aquila, Italy

2 Department of Clinical and Experimental Medicine, Rheumatology Unit, University of Perugia, 06122 Perugia, Italy

3 Division and Laboratory of Rheumatology, University of Pavia, 27100 Pavia, Italy

4 Department of Internal Medicine, Rheumatology Unit, University of Palermo, 90127 Palermo, Italy

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Arthritis Research & Therapy 2013, 15:R172  doi:10.1186/ar4359

Published: 30 October 2013



Primary Sjögren’s syndrome (pSS) is an autoimmune disorder affecting exocrine glands; however, a subgroup of pSS patients experience systemic extra-glandular involvement leading to a worsening of disease prognosis. Current therapeutic options are mainly empiric and often translated by other autoimmune diseases. In the last few years growing evidence suggests that B-cell depletion by rituximab (RTX) is effective also in pSS. Patients with early active disease appear to be those who could benefit the most from RTX. The aim of this study was to investigate the efficacy and safety of RTX in comparison to disease modifying anti-rheumatic drugs (DMARDs) in early active pSS patients.


Forty-one patients with early pSS and active disease (EULAR Sjogren’s syndrome disease activity index, ESSDAI ≥ 6) were enrolled in the study. Patients were treated with either RTX or DMARDs in two different Rheumatology centers and followed up for 120 weeks. Clinical assessment was performed by ESSDAI every 12 weeks up to week 120 and by self-reported global disease activity pain, sicca symptoms and fatigue on visual analogic scales, unstimulated saliva flow and Schirmer’s I test at week 12, 24, 48, 72, 96, and 120. Laboratory assessment was performed every 12 weeks to week 120. Two labial minor salivary gland (MSG) biopsies were obtained from all patients at the time of inclusion in the study and at week 120.


Our study demonstrated that RTX treatment results in a faster and more pronounced decrease of ESSDAI and other clinical parameters compared to DMARDs treatment. No adverse events were reported in the two groups. We also observed that RTX is able to reduce glandular infiltrate, interfere with B/T compartmentalization and consequently with the formation of ectopic lymphoid structures and germinal center-like structures in pSS-MSGs.


To our knowledge, this is the first study performed in a large cohort of early active pSS patients for a period of 120 weeks. We showed that RTX is a safe and effective agent to be employed in pSS patients with systemic, extra-glandular involvement. Furthermore, our data on pSS-MSGs provide additional biological basis to employ RTX in this disease.