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Intravenous Immunoglobulin in Idiopathic Inflammatory Myopathies: a Practical Guide for Clinical Use

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Abstract

Purpose of Review

Idiopathic inflammatory myopathies (IIM) are a complex family of autoimmune systemic disorders which often affect muscle and/or skin. IIM cause significant morbidity and mortality, but optimal treatment is uncertain. This review provides a practical guide for using intravenous immunoglobulin (IVIG) and subcutaneous immunoglobulin (SCIG) in the management of IIM, including dermatomyositis (DM), polymyositis (PM), immune-mediated necrotizing myositis (IMNM), and spontaneous inclusion body myositis (IBM), based on relevant recent literature and experience. We summarize pertinent considerations when using IVIG in special circumstances, including myositis-related dysphagia, interstitial lung disease (ILD), calcinosis cutis, and pregnant patients. This review also discusses IVIG safety, available formulations, and costs.

Recent Findings

While IVIG has been used de facto for severe IIM for over 30 years, prior clinical trials of IVIG were notably limited. Recently, however, IVIG has proven safe and effective against IIM in several high-impact publications, including a large prospective, randomized placebo-controlled phase III study in DM.

Summary

IVIG is useful against both muscular and extra-muscular manifestations in many types of IIM. It can be used as a first-line, steroid-sparring agent or as add-on to other treatments, tailored to specific clinical IIM scenarios. It is generally well-tolerated and has good safety profile, but accessibility and cost still limit its use.

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  1. Prateek C. Gandiga and Daniela Ghetie contributed equally to this work.

Authors and Affiliations

  1. Division of Rheumatology, Emory University, Atlanta, GA, USA

    Prateek C. Gandiga

  2. Division of Arthritis and Rheumatic Diseases, Oregon Health and Science University, Portland, OR, USA

    Daniela Ghetie

  3. Department of Medicine, Oregon Health and Science University, Portland, OR, USA

    Elizabeth Anderson

  4. Division of Rheumatology, University of Pittsburgh, Pittsburgh, PA, USA

    Rohit Aggrawal

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PCG notes the following disclosures: (1) Kezar Pharmaceuticals [Consultant]. DG notes the following disclosures: (1) Revolo Biotherapeutics [Consultant]. RA notes the following disclosures: (1) Mallinckrodt [Consultant, Research grant], (2) Octapharma [Consultant], (3) CSL Behring [Consultant], (4) Bristol Myers-Squibb [Consultant, Research grant], (5) EMD Serono [Consultant, Research grant], (6) Q32 [Research grant], (7) Kezar [Consultant], (8) Pfizer [Consultant, Research grant], (9) AstraZeneca [Consultant], (10) Alexion [Consultant], (11) Argenx [Consultant], (12) Boehringer Ingelheim (BI) [Consultant, Research grant], (13) Corbus [Consultant], (14) Janssen [Consultant, Research grant], (15) Kyverna [Consultant], (16) Roivant [Consultant], (17) Merck [Consultant], (18) Galapagos [Consultant], (19) Actigraph [Consultant], (20) Scipher [Consultant], (21) Horizon Therapeutics [Consultant], (22) Teva [Consultant], (23) Beigene [Consultant], (24) ANI Pharmaceuticals [Consultant], (25) Biogen [Consultant], (26) Nuvig [Consultant], (27) Capella Bioscience [Consultant], (28) CabalettaBio [Consultant]. EA has no external relationships to disclose.

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Gandiga, P.C., Ghetie, D., Anderson, E. et al. Intravenous Immunoglobulin in Idiopathic Inflammatory Myopathies: a Practical Guide for Clinical Use. Curr Rheumatol Rep 25, 152–168 (2023). https://doi.org/10.1007/s11926-023-01105-w

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