Anti-HMGCR Myopathy

Payam Mohassel, Andrew L. Mammen

Research output: Contribution to journalReview articlepeer-review

Abstract

Anti-HMGCR myopathy was first recognized and characterized in patients with a history of statin exposure and immune-mediated necrotizing myopathy. After the discovery of anti-HMGCR autoantibodies, several international groups identified and characterized more patients, expanding the phenotypic spectrum of this disease to include pediatric patients and young adults without statin exposure and those with a chronic myopathy resembling limb-girdle muscular dystrophy. We provide a summary of clinical findings, pathologic features, muscle imaging, and immunogenetic risk factors of the disease. We also discuss the current treatment strategies and approaches to monitoring the therapeutic response. Lastly, we briefly summarize the current understanding of the pathophysiology of the disease and postulate a model for autoimmunity initiation and propagation in this disease.

Original languageEnglish (US)
Pages (from-to)11-20
Number of pages10
JournalJournal of neuromuscular diseases
Volume5
Issue number1
DOIs
StatePublished - 2018
Externally publishedYes

Keywords

  • Anti-HMGCR myopathy
  • autoimmunity
  • limb-girdle muscular dystrophy
  • myositis
  • necrotizing myopathy

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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