Objective This work aims to evaluate the long-term safety of rituximab (RTX) in primary Sjögren's syndrome (pSS) to determine the safety and the efficacy of long-term treatment with B cell depleting therapy in pSS patients with active systemic disease. Methods An historical cohort study, enrolling 35 pSS patients treated with RTX, between 2008 and 2019, in a single Rheumatologic Unit was performed. When patients experienced adverse events, the treatment was suspended, and patients' data were recorded. Results The included patients were mainly female (91%) with a mean age of 54 years. During the observation, 13 patients (37.1%) suspended RTX treatment (10 cases per 100 patient-years, 95% CI: 0.061-0.17). Baseline demographics, disease characteristics, ESSDAI values, and treatment were comparable across RTX-suspended and non-suspended. Patients exposed to RTX had been followed for 35.82 ± 32.56 months, and the time of observation varied from 96 to 6 months. All the patients, except one, experienced a significant and persisting meaningful improvement of their ESSDAI (≥ 3 points) during the long-time follow-up. For the duration of the follow-up, 13 (37%) patients discontinued RTX treatment. Four out of 13 (30.8%) discontinued the treatment after the first infusion due to infusion-related reactions. During subsequent courses, the main cause of withdrawal was hypogammaglobulinemia onset (7 patients). In 2 patients, hypogammaglobulinemia was associated with severe infections. Conclusion RTX long-term administration showed to be a safe, well-tolerated, and effective treatment in patients with active systemic disease, significantly reducing ESSDAI, and the control of the disease activity last for years.
A retrospective study of long-term outcomes in 152 patients with primary Sjögren's syndrome: 25-year experience
