Safety and effectiveness of rituximab therapy in Sjögren's disease: a PORTRESS - the Portuguese registry of Sjögren's disease-based study.

introductionSjögren's Disease (SjD) is an immune-mediated disorder characterised by lymphocytic infiltration of exocrine glands and, frequently, systemic involvement. Although B-cell hyperactivity provides a rationale for using rituximab (RTX) therapy, randomised clinical trials have failed to demonstrate consistent efficacy, while real-world data suggest benefits in systemic manifestations.

objectivesTo evaluate the effectiveness and safety of RTX in patients with SjD registered in PORTRESS, the Portuguese Registry of Sjögren's disease.

methodsWe conducted a multicentre, retrospective, observational study including SjD adult patients treated with RTX and registered in PORTRESS. Demographic, clinical, immunological, and treatment data were extracted. Changes between baseline and 12 months were analysed using paired statistical tests.

resultsSixty-nine patients were analysed (87% female; mean age 55.4 ± 15.1 years). At the start of RTX, 84% were receiving concomitant immunosuppressive therapy, mainly glucocorticoids (66.7%). ESSDAI significantly decreased from 11(6-22) to 4(0-8) at 12 months (p < .001). A clinically meaningful response (ΔESSDAI ≥ 3) was achieved in 74% of patients. ESSPRI and both patient and physician global assessments also improved significantly. Median glucocorticoid dose decreased from 5.0(0-12.5) to 0(0-5.0) mg/day (p < .001). RTX was well tolerated, with mild-to-moderate adverse events reported in 13.0% of cases. Treatment was discontinued within the first 12 months in 20.3% of patients, mainly due to adverse events (7.2%).

conclusionsIn this real-world multicentre cohort, RTX demonstrated significant effectiveness, reducing disease activity and glucocorticoid use, and an acceptable safety profile in SjD patients with active systemic disease. These findings support RTX as a therapeutic option in refractory systemic disease under routine clinical conditions. Key Points • Rituximab significantly reduced systemic disease activity and glucocorticoid use in patients with Sjögren's Disease under real-world clinical conditions. • Nearly three-quarters of patients achieved a clinically meaningful ESSDAI response (≥ 3-point improvement) after 12 months of treatment. • Patient-reported outcomes (ESSPRI and global assessments) improved significantly, supporting overall clinical benefit. • Rituximab showed an acceptable safety profile, with mostly mild-to-moderate adverse events.