Efficacy and safety of intravenous and subcutaneous immunoglobulin therapy in idiopathic inflammatory myopathy: A systematic review and meta-analysis

Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, India. Electronic address: rpgaiims512@gmail.com. Department of Medicine and Microbiology, All India Institute of Medical Sciences, New Delhi, India. Department of Mathematics and Statistics, Aliah University, Kolkata, India. Department of Rheumatology, All India Institute of Medical Sciences, New Delhi, India. Department of Neurology, Amsterdam UMC, University of Amsterdam, Amsterdam Neuroscience, PO Box 22660, 1100DD Amsterdam, the Netherlands. Electronic address: a.j.kooi@amsterdamumc.nl. Department of Neurology, Amsterdam UMC, University of Amsterdam, Amsterdam Neuroscience, PO Box 22660, 1100DD Amsterdam, the Netherlands. Electronic address: j.lim@amsterdamumc.nl. Department of Neurology, Amsterdam UMC, University of Amsterdam, Amsterdam Neuroscience, PO Box 22660, 1100DD Amsterdam, the Netherlands. Electronic address: j.raaphorst@amsterdamumc.nl. Emergency Medicine, AziendaOspedaliera Marche Nord, Fano, PU 61032, Italy. Clinica Medica, Department of Clinical and Molecular Sciences, Polytechnic University of Marche, Torrette di Ancona 60020, Italy. Electronic address: m.g.danieli@univpm.it.

Autoimmunity reviews. 2021;:102997
Abstract
OBJECTIVE To perform a systematic review and meta-analysis on the efficacy and safety of intravenous (IVIg) and subcutaneous (SCIg) immunoglobulin (Ig) therapy in the treatment of idiopathic inflammatory myopathy (IIM) and juvenile dermatomyositis (JDM). METHODS PubMed, Embase and SCOPUS were searched to identify studies on Ig therapy in patients with IIM and/or JDM (2010-2020). Outcome measures were complete response (CR) or partial response (PR) in terms of muscle power and extramuscular disease activity measures on the International Myositis Assessment and Clinical Studies Group (IMACS) core set domains. RESULTS Twenty-nine studies were included (n = 576, 544 IIM, 32 JDM). Muscle power PR with pooled Ig therapy was 88.5% (95% confidence interval (CI): 80.6-93.5, n = 499) and PR with SCIg treatment was 96.61% (95% CI: 87.43-99.15, n = 59). Pooled PR with first-line use of IVIg was 77.07% (95% CI: 61.25-92.89, n = 80). Overall, mean time to response was 2.9 months (95% CI: 1.9-4.1). Relapse was seen in 22.76% (95% CI: 14.9-33). Studies on cutaneous disease activity and dysphagia showed significant treatment responses. Glucocorticoid and immunosuppressant sparing effect was seen in 40.9% (95% CI: 20-61.7) and 42.2% (95% CI: 20.4-64.1) respectively. Ig therapy was generally safe with low risk of infection (1.37%, 95% CI: 0.1-2.6). CONCLUSIONS Add-on Ig therapy improves muscle strength in patients with refractory IIM, but evidence on Ig therapy in new-onset disease and extramuscular disease activity is uncertain.
Study details
Study Design : Systematic Review
Language : eng
Credits : Bibliographic data from MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine