Biologic Therapy for Systemic Sclerosis: A Systematic Review

Objective. Biologic agents are increasingly used in the rheumatic diseases. Their role in patients with systemic sclerosis (SSc) is uncertain. Our aim was to evaluate the effectiveness and safety of biologic agents in SSc. We review the evidence for the use of biologic agents to improve inflammatory arthritis, disability, and skin score, and we review adverse effects with biologic agents in patients with SSc. Methods. A systematic literature review was performed to identify studies evaluating the use of biologic agents in SSc. Medline, Embase, CINAHL, and Cochrane Database of Systematic Reviews were searched. A standardized abstraction form was used to extract biologic agent, study design, sample size, treatment effect, and adverse effects. Results. A total of 23 studies from 1413 citations were evaluated. Three studies evaluated infliximab, 3 evaluated etanercept, 3 evaluated antithymocyte globulin, 3 evaluated imatinib, 6 evaluated rituximab, and 1 study each evaluated interferon-gamma (IFN-gamma), IFN-alpha, relaxin, delipidated, deglycolipidated Mycobacterium vaccae, human anti-transforming growth factor 131 antibody, and oral type I collagen. Studies of etanercept and infliximab suggest improvements in inflammatory arthritis and Health Assessment Questionnaire Disability Index (HAQ-DI). None of the other biologic agents demonstrated reproducible, statistically significant improvements in joint count, HAQ-DI, or skin score. Conclusion. Anti-tumor necrosis factor-a agents may improve inflammatory arthritis and disability in SSc. The effect on skin score is uncertain. Adequately powered trials are needed to evaluate efficacy, and longitudinal studies are needed to evaluate longterm safety of these agents in SSc. (First Release Nov 1 2010; J Rheumatol 2011;38:289-96; doi:10.3899/jrheum.100361)