Becker Muscular Dystrophy Accompanied by Anti-HMGCR Antibody-positive Immune-mediated Necrotizing Myopathy

Intern Med. 2023 May 1;62(9):1345-1349. doi: 10.2169/internalmedicine.0202-22. Epub 2022 Sep 21.

Abstract

Becker muscular dystrophy (BMD) is an X-linked neuromuscular disease characterized by progressive muscle weakness that currently has no cure. Immune-mediated necrotizing myopathy (IMNM) is a type of autoimmune inflammatory myopathy characterized by proximal muscle weakness that is treated with immunosuppressive therapy. We herein report a patient diagnosed with BMD complicated with IMNM by a pathological analysis. Notably, the patient had an elevated serum anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibody level. Oral glucocorticoid and methotrexate treatment partially improved the muscle weakness with decreased levels of serum creatine kinase. An accurate diagnosis is important for therapeutic decisions in these complicated cases.

Keywords: Becker muscular dystrophy; anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase antibody; immune-mediated necrotizing myopathy.

MeSH terms

  • Autoantibodies
  • Autoimmune Diseases* / complications
  • Autoimmune Diseases* / diagnosis
  • Humans
  • Muscle Weakness / etiology
  • Muscular Diseases* / pathology
  • Muscular Dystrophy, Duchenne* / complications
  • Muscular Dystrophy, Duchenne* / drug therapy
  • Myositis* / complications
  • Myositis* / diagnosis
  • Myositis* / drug therapy
  • Necrosis

Substances

  • Autoantibodies
  • HMGCR protein, human