Spectrum of immune-mediated necrotizing myopathies and their treatments

Iago Pinal-Fernandez, Andrew L. Mammen

Research output: Contribution to journalReview article

Abstract

Purpose of review This review aims to describe the spectrum of clinical, histological, and serological features in patients with immune-mediated necrotizing myopathies (IMNMs). Recent findings Autoantibodies recognizing the signal recognition particle (SRP) or 3-hydroxy-3-methylglutaryl-CoA reductase (HMGCR) define two unique subtypes of necrotizing myositis patient with distinct clinical features. For example, the major histocompatibility class II human leukocyte antigen allele DRB1∗11:01 is a strong immunogenetic risk factor for developing anti-HMGCR autoantibodies whereas B∗5001 and DQA1∗0104 are over-represented in patients with anti-SRP autoantibodies. Furthermore, statin exposure is a risk factor only for anti-HMGCR autoantibodies. And while skeletal muscle involvement is predominant in most patients with both autoantibodies, lung involvement appears in ∼20% of anti-SRP-positive patients but is more rare in anti-HMGCR-positive patients. Of note, ∼20% of anti-SRP and anti-HMGCR positive patients have significant lymphocytic infiltrates on muscle biopsy and thus would not be formally categorized as having IMNM; aside from this, these patients are clinically indistinguishable from other patients with the same autoantibody profile. Summary Anti-SRP and anti-HMGCR autoantibodies define unique populations of IMNM patients. It may be more appropriate to subtype myositis patients based on these autoantibodies than on their muscle biopsy features.

Original languageEnglish (US)
Pages (from-to)619-624
Number of pages6
JournalCurrent Opinion in Rheumatology
Volume28
Issue number6
DOIs
Publication statusPublished - Oct 1 2016

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Keywords

  • 3-hydroxy-3-methylglutaryl-CoA reductase
  • Autoantibody
  • Autoimmunity
  • Immune-mediated necrotizing myopathy
  • Myositis
  • Signal recognition particle

ASJC Scopus subject areas

  • Rheumatology

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