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Update on the Diagnostic and Therapeutic Landscape of Sporadic Inclusion Body Myositis

  • Neuromuscular Disorders (C Fournier, Section Editor)
  • Published:
Current Treatment Options in Neurology Aims and scope Submit manuscript

Abstract

Purpose of the review

The purpose of this paper is to review the recent findings that pertain to the diagnosis and treatment of sporadic inclusion body myositis.

Recent findings

New evidence highlighting lack of correlation between the presence of cytosolic 5′-nucleotidase 1A antibody with any of the clinical features, or laboratory findings in inclusion body myositis. New studies emphasizing heterogeneity and showing separation of inclusion body myositis patients into separate trajectories. The failure of bimagrumab and arimoclomol as potential treatments of inclusion body myositis and the mixed results of the sirolimus trial in sporadic inclusion body myositis.

Summary

A significant gap exists in understanding the heterogeneity of sporadic inclusion body myositis. Recent evidence suggests that cytosolic 5′-nucleotidase 1A antibody does not provide significant clinical or laboratory differentiation between antibody-positive and negative patients. Despite recent failures in the clinical trials of arimoclomol and bimagrumab, sirolimus showed mixed results, and a larger definitive trial is needed.

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Correspondence to Hani Kushlaf MD.

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Conflict of Interest

Dr. Kushlaf has served as a consultant on advisory boards for Alexion Pharmaceuticals, Catalyst Pharmaceuticals, Sanofi Genzyme, PTC therapeutics, and Takeda. Dr. Kushlaf serves on the speaker bureaus of Akcea, Catalyst Pharmaceuticals, and Sanofi Genzyme. None of these disclosures was relevant to this editorial.

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Kushlaf, H. Update on the Diagnostic and Therapeutic Landscape of Sporadic Inclusion Body Myositis. Curr Treat Options Neurol 23, 27 (2021). https://doi.org/10.1007/s11940-021-00681-5

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