Clinical features of anti-mitochondrial M2 antibody-positive myositis: case series of 17 patients

J Neurol Sci. 2022 Nov 15;442:120391. doi: 10.1016/j.jns.2022.120391. Epub 2022 Aug 28.


Objective: In 2012, a large number of myositis cases with anti-mitochondrial M2 (AMA-M2) antibody, which had well been known as the serological hallmark for primary biliary cholangitis (PBC), were reported in Japan. Recently, some case series from Japan, France, America, China and India have shown that approximately 2.5% to 19.5% of patients with myositis have AMA-M2 antibody. The objective of this study was to clarify the prevalence, clinical features, treatment outcome, and severity determinants of AMA-M2 positive myositis.

Methods: This study was a multicenter observational study. We enrolled patients who were diagnosed with myositis during a ten-year period between 2012 and 2021.

Results: Of the total of 185 patients with inflammatory myopathy, 17 patients were positive for AMA-M2 antibody. The typical symptoms were weakness mainly involving paravertebral muscles, weight loss, respiratory failure, and cardiac complications. Thirteen of the 17 patients had cardiac complications. A strong correlation was found between respiratory failure and modified Rankin Scale (mRS) score. A strong correlation was also found between respiratory failure and body weight, indicating that weight loss can be an indicator of potential progression of respiratory failure. Six of the 17 patients were complicated by malignancy.

Conclusions: This study showed significant correlations between % vital capacity (VC), body mass index (BMI), and mRS score in patients with AMA-M2-positive myositis. Immunotherapy often improved CK level and respiratory dysfunction. We therefore propose that %VC and BMI should be monitored as disease indicators in treatment of AMA-M2-positive myositis.

Keywords: Anti-mitochondrial M2 antibody; Cardiac complication; Chronic clinical course; Inflammatory myopathy; Myositis; Respiratory failure; Weight loss.

Publication types

  • Observational Study
  • Multicenter Study

MeSH terms

  • Autoantibodies
  • Humans
  • Liver Cirrhosis, Biliary* / diagnosis
  • Myositis* / diagnosis
  • Myositis* / therapy
  • Respiratory Insufficiency*
  • Weight Loss


  • Autoantibodies