Efficacy and Safety of Immunosuppressive Therapy in Connective Tissue Disease-Related Immune Thrombocytopenia: a Systematic Review and Meta-Analysis

Background: Connective tissue diseases (CTDs) are a group of autoimmune disorders in which immune thrombocytopenia (ITP) represents a common and important manifestation. Current CTD-ITP management mainly relies on glucocorticoids and immunosuppressive agents.Objectives: This systematic review and meta-analysis aims to evaluate the efficacy and safety of immunosuppressive agents in patients with CTD-ITP.Design: Systematic review and meta-analysis.Methods: This study was conducted using eight databases up to July 22, 2024. Observational studies and experimental trials with relevant efficacy and safety data were included. Methodological quality were evaluated using the Newcastle-Ottawa Scale and the ROBINS-I tool. The Mantel-Haenszel formula with a random effect model was employed to estimate the overall effect size. Subgroup analyses were performed based on the study characteristics, clinical features, and treatment regimen.Results: Through integrating 24 studies (1 single-arm clinical trial, 1 case-control, and 22 cohort studies) involving 775 CTD-ITP patients, the pooled estimates of the optimal overall and complete response rate for immunosuppressive therapy were 82% (95% CI: 75-88) and 64% (95% CI: 56-72), with stable results after sensitivity analysis. The combined side-effect incidence was 31% (95% CI: 24-39). The pooled relapse rate was 30% (95% CI: 18-43) among 403 immunosuppressive therapy responders.Conclusion: Immunosuppressive treatments exhibited favorable efficacy and safety in CTD-ITP patients. Future larger-scale multicenter studies are needed.