Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
Review
. 2023 Mar 20:14:1123380.
doi: 10.3389/fneur.2023.1123380. eCollection 2023.

Pediatric immune-mediated necrotizing myopathy

Chen-Hua Wang  1   2 , Wen-Chen Liang  2   3   4
Affiliations
Review

Pediatric immune-mediated necrotizing myopathy

Chen-Hua Wang et al. Front Neurol. .

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.

PubMed Disclaimer

Conflict of interest statement

The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.

References

    1. Bohan A, Peter JB. Polymyositis and dermatomyositis (first of two parts). N Engl J Med. (1975) 292:344–7. doi: 10.1056/NEJM197502132920706 - DOI - PubMed
    1. Griggs RC, Askanas V, DiMauro S, Engel A, Karpati G, Mendell JR, et al. . Inclusion body myositis and myopathies. Ann Neurol. (1995) 38:705–13. doi: 10.1002/ana.410380504 - DOI - PubMed
    1. Mariampillai K, Granger B, Amelin D, Guiguet M, Hachulla E, Maurier F, et al. . Development of a new classification system for idiopathic inflammatory myopathies based on clinical manifestations and myositis-specific autoantibodies. JAMA Neurol. (2018) 75:1528–37. doi: 10.1001/jamaneurol.2018.2598, PMID: - DOI - PMC - PubMed
    1. Christopher-Stine L, Casciola-Rosen LA, Hong G, Chung T, Corse AM, Mammen AL. A novel autoantibody recognizing 200-Kd and 100-Kd proteins is associated with an immune-mediated necrotizing myopathy. Arthritis Rheum. (2010) 62:2757–66. doi: 10.1002/art.27572, PMID: - DOI - PMC - PubMed
    1. Mammen AL, Chung T, Christopher-Stine L, Rosen P, Rosen A, Doering KR, et al. . Autoantibodies against 3-hydroxy-3-methylglutaryl-coenzyme a reductase in patients with statin-associated autoimmune myopathy. Arthritis Rheum. (2011) 63:713–21. doi: 10.1002/art.30156, PMID: - DOI - PMC - PubMed

LinkOut - more resources