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Treatment of Immune-Mediated Necrotizing Myopathy

  • Other CTD: Inflammatory Myopathies (J Milisenda, Section Editor)
  • Published:
Current Treatment Options in Rheumatology Aims and scope Submit manuscript

Abstract

Purpose of Review

Immune-mediated necrotizing myositis (IMNM) is a rare autoimmune disorder characterized by proximal muscle weakness, elevated creatine kinase levels, and necrosis of muscle fibers. While the exact pathogenesis of IMNM remains unknown, anti-HMGCR and anti-SRP autoantibodies are associated with different predisposing factors, clinical manifestations, and severity of the disease and are believed to correspond to two pathogenically distinct entities. The cornerstone treatment for IMNM is a combination of glucocorticoids and steroid-sparing agents. Therapeutic strategies aimed at decreasing the half-life of endogenous autoantibodies, such as intravenous immunoglobulin (IVIG), or reducing their production, such as rituximab, have shown promise as powerful treatments. In severe cases, combining IVIG and rituximab can have synergistic effects.

Recent Findings

Previous studies suggested that complement dysregulation may be involved in the pathogenesis of IMNM. However, a recent phase 2 clinical trial evaluating the effectiveness of zilucoplan, a C5 inhibitor, failed to show efficacy in IMNM.

Summary

In this review, we aim to provide a comprehensive review of IMNM focusing on the current evidence regarding treatment options for this condition. Our goal is to present an up-to-date overview of the current state of therapeutics on IMNM and highlight potential areas for future investigation.

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Funding

This study was funded, in part, by the Intramural Research Program of the National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health.

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Authors and Affiliations

  1. Muscle Disease Unit, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, 50 South Drive, Room 1141, Building 50, MSC 8024, Bethesda, MD, 20892, USA

    Sandra Amara Ogbonnaya-Whittlesey, Dale Kobrin, Maria Casal-Dominguez, Andrew L. Mammen & Iago Pinal-Fernandez

  2. Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Maria Casal-Dominguez, Andrew L. Mammen & Iago Pinal-Fernandez

Authors
  1. Sandra Amara Ogbonnaya-Whittlesey
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  2. Dale Kobrin
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  3. Maria Casal-Dominguez
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  4. Andrew L. Mammen
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  5. Iago Pinal-Fernandez
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Corresponding authors

Correspondence to Andrew L. Mammen or Iago Pinal-Fernandez.

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Conflict of Interest

Sandra Amara Ogbonnaya-Whittlesey declares that she has no conflict of interest. Dale Kobrin declares that he has no conflict of interest. Maria Casal-Dominguez declares that she has no conflict of interest. Andrew L. Mammen declares that he has no conflict of interest. Iago Pinal-Fernandez declares that he has no conflict of interest.

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Ogbonnaya-Whittlesey, S.A., Kobrin, D., Casal-Dominguez, M. et al. Treatment of Immune-Mediated Necrotizing Myopathy. Curr Treat Options in Rheum 9, 168–178 (2023). https://doi.org/10.1007/s40674-023-00210-2

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