Inflammatory Myopathies

Georgios Manousakis, MD, FAAN Muscle and Neuromuscular Junction Disorders p. 1643-1662 December 2022, Vol.28, No.6 doi: 10.1212/CON.0000000000001179
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PURPOSE OF REVIEW This article outlines the salient clinical, serologic, electrophysiologic, imaging, and histopathologic findings and treatment options for the idiopathic inflammatory myopathies, including those related to immune checkpoint inhibitors and SARS-CoV-2.

RECENT FINDINGS The classification of idiopathic inflammatory myopathies has improved with the integration of myositis-specific antibodies and histopathologic findings. Characteristic features of immune checkpoint inhibitor–related myositis have been identified, allowing early recognition and treatment of the syndrome. The COVID-19 pandemic has had a profound impact on the care of patients with idiopathic inflammatory myopathies, and several mechanisms of virus-related muscle injury have been proposed.

SUMMARY A comprehensive evaluation including clinical examination, EMG, imaging, antibody testing, muscle biopsy, and cancer screening, when appropriate, can lead to an earlier accurate diagnosis and an individualized treatment approach for patients with idiopathic inflammatory myopathies.

Address correspondence to Dr Georgios Manousakis, 420 Delaware St SE, MMC 295, Minneapolis, MN 55419, [email protected].

RELATIONSHIP DISCLOSURE: Dr Manousakis has received personal compensation in the range of $500 to $4999 for serving on a scientific advisory or data safety monitoring board for Alexion Pharmaceuticals, Inc, argenx, and Stealth BioTherapeutics Inc. The institution of Dr Manousakis has received research support from the Greg Marzolf Jr. Foundation.

UNLABELED USE OF PRODUCTS/INVESTIGATIONAL USE DISCLOSURE: Dr Manousakis discusses the unlabeled/investigational use of several immunosuppressive agents for the treatment of immune/inflammatory myopathies.

© 2022 American Academy of Neurology.