Rituximab treatment for rhupus syndrome: a systematic literature review

Introduction: Rhupus is a poorly described and underdiagnosed disease in which features of both rheumatoid arthritis (RA) and systemic lupus erythematous (SLE) appear in the same patient. Herein, we present a systematic literature review to analyze the clinical, laboratory and radiological data of all reported rhupus patients treated with rituximab (RTX). Methods: This systematic review followed the preferred reporting items for systematic reviews guidelines. Eligible articles were searched in Medline, Embase, and Cochrane Library. We analyzed cohort studies, case series and case reports. Results: Four papers were finally selected for analysis. The studies were published between 2012 and 2022. Overall, 29 patients were included. All rhupus patients were previously treated with conventional Disease-Modifying Antirheumatic Drugs (DMARDs) and RTX was the first biologic DMARD prescribed in 22 patients. A significant reduction in mean the 28 Disease-activity-score (DAS28) from baseline to the end of follow-up was observed in all the studies. Mean DAS28 decreased from 5.65 to 3.65. The clinical activity of SLE was evaluated in 2 studies using The Systemic Lupus Erythematosus Disease Activity Index (SLEDAI). Overall, mean SLEDAI decreased from 6.05 at baseline to 2.66 at the end of follow-up Based on the ultrasonographic (US) disease activity assessment performed one study, US count scores progressively reduced, indicating that a US response was sustained after RTX treatment. Overall, only two severe adverse events were noted. Conclusion: In the light of this literature systematic review, RTX seems to be effective and safe in rhupus syndrome.