Muscle pathological features and extra-muscle involvement in idiopathic inflammatory myopathies with anti-mitochondrial antibody.

OBJECTIVE Anti-mitochondrial antibodies (AMAs) can be detected in some idiopathic inflammatory myopathy (IIM) patients. We aimed to investigate the clinical features of IIM patients with AMAs. METHODS We retrospectively analysed 1,167 consecutive patients with IIM for AMA-associated myositis and compared them to age- and gender-matched AMA-negative IIM patients. RESULTS Twenty-nine patients (2.5%) were identified with AMA-positive myositis; eight of them had primary biliary cholangitis (PBC). There were no significant differences in skin rash, dysphagia, interstitial lung disease, and muscle strength between AMA-positive patients and AMA-negative patients. Of 23 cases, 12 (52.2%) showed immune-mediated necrotizing myopathy (IMNM)-like pathological features. amongst AMA-positive patients, 11 of 16 patients with isolated anti-AMAs were classified as IMNM which was significantly higher than that of patients with coexistent anti-AMAs and myositis-specific antibodies (p = 0.026). Moreover, subclinical cardiac involvement was significantly more common in AMA-positive patients than in AMA-negative patients (21/29 VS 33/116, p<0.001). In addition, patients without PBC had a significantly higher incidence of abnormal echocardiography findings than that of patients with PBC (p = 0.009). Patients without heart abnormalities took significantly less time to achieve disease remission and prednisone tapering to <10 mg than patients with heart abnormalities (p = 0.000 and p = 0.001, respectively). CONCLUSIONS IMNM was a major histopathological finding in IIM patients with isolated AMAs. AMAs were significantly associated with subclinical cardiac involvement in IIM. PBC seemed to be a protective factor for abnormal echocardiography findings in AMA-positive patients. Patients without heart involvement took less time to achieve disease remission and prednisone tapering off.