Inflammatory myopathies

Anti-mitochondrial M2 antibody links to cardiac involvement and immune-mediated inflammatory myopathy-like pathology in myositis

Rheumatology (Oxford)Published Historic prospective cohort study

Authors' conclusion

AMA-M2 positivity defines an IIM subgroup with significant cardiac involvement and an immune-mediated inflammatory muscle histology, but not necessarily worse long-term survival.

Study context

Population
Idiopathic inflammatory myopathies (IIM) patients hospitalized at Peking Union Medical College Hospital, 2008–2020
Participants
987 patients
Exposure
AMA-M2-positive vs AMA-M2-negative IIM
  • AMA-M2-positiven = 55
Outcome
Cardiac involvement throughout disease course
Methodological detailssingle-centre, registry-based (Prospective Registry Of MyositIS (PROMIS)), multivariate Cox regression
  • Single-centre
  • Registry-based (PROMIS)
  • Multivariate Cox regression

Main finding

As reported in the abstract

GroupValueArrhythmiasHFPulmonary hypertension
AMA-M2-positive60%56%44%31%
AMA-M2-negative12.9%

Value

AMA-M2-positive60%
AMA-M2-negative12.9%

Arrhythmias

AMA-M2-positive56%
AMA-M2-negative— not stated in the abstract

HF

AMA-M2-positive44%
AMA-M2-negative— not stated in the abstract

Pulmonary hypertension

AMA-M2-positive31%
AMA-M2-negative— not stated in the abstract
  • AMA-M2-positive vs AMA-M2-negative: P < 0.001
AMA-M2 positivity as independent risk factor for cardiac involvement (multivariate Cox), HR
3.156, P < 0.001

Observational data: associations, not effects.

Further findings

PM rate

P < 0.001

Long-term survival

P = 0.86

OutcomeAMA-M2-positiveAMA-M2-negativeP value
Baseline gamma-glutamyl transferase78.035.0P < 0.001
Baseline alkaline phosphatase85.064.0P < 0.001

Biopsy findings

Some muscle biopsies showed features consistent with immune-mediated necrotizing myopathy; cardiac biopsies showed structural degeneration with minimal inflammation; liver biopsies confirmed early-stage primary biliary cholangitis (PBC)

Original publication

Anti-mitochondrial M2 antibody links to cardiac involvement and immune-mediated inflammatory myopathy-like pathology in myositis. Rheumatology (Oxford). Published 2 January 2026. doi:10.1093/rheumatology/keaf670

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