Anti-mitochondrial autoantibodies are associated with cardiomyopathy, dysphagia, and features of more severe disease in adult-onset myositis
Open Access
- 13 April 2021
- journal article
- research article
- Published by Springer Science and Business Media LLC in Clinical Rheumatology
- Vol. 40 (10), 4095-4100
- https://doi.org/10.1007/s10067-021-05730-7
Abstract
We analyzed the prevalence of anti-mitochondrial autoantibodies (AMA) in adult- and juvenile-onset myositis longitudinal cohorts and investigated phenotypic differences in myositis patients with AMA. We screened sera from myositis patients including 619 adult- and 371 juvenile-onset dermatomyositis (DM, JDM), polymyositis (PM, JPM), inclusion body myositis (IBM), or amyopathic DM patients and from healthy controls, including 164 adults and 92 children, for AMA by ELISA. Clinical characteristics were compared between myositis patients with and without AMA. AMA were present in 5% of adult myositis patients (16 of 216 DM, 10 of 222 PM, 4 of 140 IBM, 1 of 19 amyopathic DM), 1% of juvenile myositis patients (3 of 302 JDM, 1 of 25 JPM), and 1% of both adult and juvenile healthy controls. In patients with adult-onset myositis, AMA were associated with persistent muscle weakness, Raynaud’s phenomenon, dysphagia, and cardiomyopathy. Adult myositis patients with AMA may have more severe or treatment refractory disease, as they more frequently received glucocorticoids and intravenous immunoglobulin. In juvenile myositis, children with AMA often had falling episodes and dysphagia, but no other clinical features or medications were significantly associated with AMA. AMA are present in 5% of adult myositis patients and associated with cardiomyopathy, dysphagia, and other signs of severe disease. The prevalence of AMA is not increased in patients with juvenile myositis compared to age-matched healthy controls. Our data suggest that the presence of AMA in adult myositis patients should prompt screening for cardiac and swallowing involvement. Key Points • Approximately 5% of a large North American cohort of adult myositis patients have anti-mitochondrial autoantibodies. • Adults with anti-mitochondrial autoantibodies often have chronic weakness, Raynaud’s, dysphagia, cardiomyopathy, and more severe disease. • Anti-mitochondrial autoantibodies are rare in juvenile myositis and not associated with a specific clinical phenotype.This publication has 16 references indexed in Scilit:
- Large‐scale characterization study of patients with antimitochondrial antibodies but nonestablished primary biliary cholangitisJournal of Hepatology, 2016
- The Prevalence of Individual Histopathologic Features Varies according to Autoantibody Status in Muscle Biopsies from Patients with Dermatomyositis.2015
- The Prevalence of Individual Histopathologic Features Varies according to Autoantibody Status in Muscle Biopsies from Patients with DermatomyositisThe Journal of Rheumatology, 2015
- The Clinical Phenotypes of the Juvenile Idiopathic Inflammatory MyopathiesMedicine, 2013
- Endothelial mitochondria and heart diseaseCardiovascular Research, 2010
- Juvenile dermatomyositis and other idiopathic inflammatory myopathies of childhoodThe Lancet, 2008
- Endogenous Endothelial Nitric Oxide Synthase–Derived Nitric Oxide Is a Physiological Regulator of Myocardial Oxygen ConsumptionCirculation Research, 1999
- Inclusion body myositis and myopathiesAnnals of Neurology, 1995
- Myopathy with mitochondrial alterations in patients with primary biliary cirrhosis and antimitochondrial antibodiesArthritis & Rheumatism, 1993
- Polymyositis and DermatomyositisThe New England Journal of Medicine, 1975