Pediatric immune-mediated necrotizing myopathy

Chen-Hua Wang; Wen-Chen Liang

doi:10.3389/fneur.2023.1123380

Pediatric immune-mediated necrotizing myopathy

Front Neurol. 2023 Mar 20:14:1123380. doi: 10.3389/fneur.2023.1123380. eCollection 2023.

Authors

Chen-Hua Wang^{1

2}, Wen-Chen Liang^{2

3

4}

Affiliations

¹ Department of Pediatrics, Kaohsiung Municipal Hsiao-Kang Hospital, Kaohsiung, Taiwan.
² Department of Pediatrics, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan.
³ Department of Pediatrics, School of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.
⁴ Graduate Institute of Clinical Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan.

Abstract

Immune-mediated necrotizing myopathy (IMNM) is a type of inflammatory myopathy. Most patients with IMNM produce anti-3-hydroxy-3-methylglutaryl coenzyme A reductase or anti-signal-recognition particle autoantibodies. IMNM is much rarer in children than in adults. We conducted this mini review focusing on pediatric IMNM to present current evidence regarding its epidemiology, clinical characteristics, diagnosis, and treatment. Our findings indicate that pediatric IMNM often causes severe muscle weakness and is refractory to corticosteroids alone. Furthermore, delayed diagnosis is common because of the clinicopathological similarity between IMNM and inherited myopathy. Raising awareness regarding pediatric IMNM may facilitate early diagnosis and effective treatment.

Keywords: HMGCR; SRP; idiopathic inflammatory myopathy; immune-mediated necrotizing myopathy; juvenile myositis.

Publication types

Review