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Khan NAJ, Khalid S, Ullah S, et al. Necrotizing Autoimmune Myopathy: A Rare Variant of Idiopathic Inflammatory Myopathies. J Investig Med High Impact Case Rep. 2017;5(2):2324709617709031doi: 10.1177/2324709617709031.
Khan, N. A. J., Khalid, S., Ullah, S., Malik, M. U., & Makhoul, S. (2017). Necrotizing Autoimmune Myopathy: A Rare Variant of Idiopathic Inflammatory Myopathies. Journal of investigative medicine high impact case reports, 5(2), 2324709617709031. https://doi.org/10.1177/2324709617709031
Khan, Noman Ahmed Jang, et al. "Necrotizing Autoimmune Myopathy: A Rare Variant of Idiopathic Inflammatory Myopathies." Journal of investigative medicine high impact case reports vol. 5,2 (2017): 2324709617709031. doi: https://doi.org/10.1177/2324709617709031
Khan NAJ, Khalid S, Ullah S, Malik MU, Makhoul S. Necrotizing Autoimmune Myopathy: A Rare Variant of Idiopathic Inflammatory Myopathies. J Investig Med High Impact Case Rep. 2017 Jun 14;5(2):2324709617709031. doi: 10.1177/2324709617709031. eCollection 2017. PMID: 28660228; PMCID: PMC5476327.
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J Investig Med High Impact Case Rep. 2017 Jun 14;5(2):2324709617709031. doi: 10.1177/2324709617709031. eCollection 2017.

Necrotizing Autoimmune Myopathy: A Rare Variant of Idiopathic Inflammatory Myopathies.

Journal of investigative medicine high impact case reports

Noman Ahmed Jang Khan, Shaza Khalid, Saad Ullah, Muhammad Umair Malik, Samer Makhoul

Affiliations

  1. Temple University, Philadelphia, PA, USA.
  2. Conemaugh Memorial Hospital, Johnstown, PA, USA.

PMID: 28660228 PMCID: PMC5476327 DOI: 10.1177/2324709617709031

Abstract

Idiopathic inflammatory myopathies are an unusual group of myopathies with annual incidence of 1 in 100 000 people in the United States. Necrotizing autoimmune myopathy comprises only 16% of this group. It usually presents with severe proximal weakness, lower extremity weakness, and severe fatigue while very rarely does it present with dysphagia and respiratory muscle weakness. Statin use, cancer, and connective tissue disorder are the usual associated risk factors. Anti-signal recognition particle and 3-hydroxy-3-methylglutaryl-coenzyme A reductase are the 2 most common autoantibodies associated with necrotizing autoimmune myopathy. In this article, we present a very rare case of a 66-year-old male who presented with shortness of breath and dysphagia requiring intubation and ventilator support. Creatine kinase was 23 000, myoglobin was 7000, and ANA was positive. All other autoimmune and infectious workup including Lyme disease was unremarkable. Muscle biopsy turned out remarkable for necrotizing myopathy. No evidence of statin use, active malignancy, or connective tissue disease was found. He was treated with high-dose corticosteroids and a short course of intravenous immunoglobulin with very mild improvement in symptoms. Anti-signal recognition particle and 3-hydroxy-3-methylglutaryl-coenzyme A reductase could not be performed as the patient refused to pursue further medical testing. This is a very rare case of idiopathic inflammatory myopathy presenting with bulbar and respiratory muscle weakness requiring ventilator support.

Keywords: muscle necrosis; myopathy; regenerating muscle fibers

Conflict of interest statement

Declaration of Conflicting Interests: The author(s) declared no potential conflicts of interest with respect to the research, authorship, and/or publication of this article.

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