Idiopathic inflammatory myopathies with anti-mitochondrial antibodies: Clinical features and treatment outcomes in a Chinese cohort

Neuromuscul Disord. 2019 Jan;29(1):5-13. doi: 10.1016/j.nmd.2018.11.004. Epub 2018 Nov 22.


Anti-mitochondrial antibodies, the hallmark of primary biliary cirrhosis, have been detected in many patients with idiopathic inflammatory myopathies and these anti-mitochondrial-antibody-associated idiopathic inflammatory myopathies frequently show unique characteristics. We detected anti-mitochondrial antibodies in Chinese idiopathic inflammatory myopathy and summarized the clinical findings of these anti-mitochondrial-antibody-positive patients. Of 136 patients, seven (5.15%) were found to be anti-mitochondrial-antibody-positive. Primary biliary cirrhosis was present in 2 of these 7 patients, chronic disease duration in 2 patients and asymmetrical muscle weakness in 4 patients. The mean disease course was 8.58 months, and the mean creatine kinase level was 2256.53 U/L. Myositis-specific antibodies were found in 3 patients. According to clinical features and muscle histopathological findings, 3 patients were classified as dermatomyositis, 2 as possible polymyositis and 2 as necrotizing autoimmune myopathy. Of the 6 anti-mitochondrial-antibody-positive patients receiving follow-ups of 12-83 months, they all showed marked clinical improvement. Our study indicates that anti-mitochondrial antibodies are relatively rare in Chinese idiopathic inflammatory myopathy patients. These patients generally show various clinical features and have favorable treatment outcomes. Anti-mitochondrial antibody testing may be helpful to confirm the diagnosis of idiopathic inflammatory myopathy, especially in patients with atypical manifestations.

Keywords: Anti-mitochondrial antibodies; Idiopathic inflammatory myopathies; Muscle pathology.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adrenal Cortex Hormones / therapeutic use
  • Adult
  • Aged
  • Autoantibodies / blood*
  • Biomarkers / blood
  • China
  • Cohort Studies
  • Female
  • Humans
  • Liver Cirrhosis, Biliary / diagnosis
  • Liver Cirrhosis, Biliary / immunology
  • Liver Cirrhosis, Biliary / pathology
  • Liver Cirrhosis, Biliary / therapy
  • Male
  • Middle Aged
  • Mitochondria / immunology*
  • Muscles / pathology
  • Myositis / diagnosis
  • Myositis / immunology*
  • Myositis / pathology
  • Myositis / therapy*


  • Adrenal Cortex Hormones
  • Autoantibodies
  • Biomarkers