Session Type: ACR Poster Session C
Session Time: 9:00AM-11:00AM
Background/Purpose: diverse forms of cardiac involvement (CI) have been described in inflammatory myopathies (IM), and is a frequent cause of death in these diseases
Methods: A multicenter retrospective study from the REMICAM registry was performed. All patients were diagnosed with IM according to Bohan and Peter criteria, followed between Jan 1980 and Dec 2014 and classified into 7 different clinical subgroups: primary dermatomyositis (DM), primary polymyositis (PM), juvenile dermatomyositis (JuvM), cancer associated myositis (CAM), overlap myositis (OM), inclusion body myositis (IBM) and necrotizing myositis (NM). CI was defined as the presence of cardiac arrhythmia (EKG +/-Holter), myocarditis and/or pericarditis (echocardiography). Descriptive statistics, univariate and multivariate analysis were performed. Kaplan Meyer curves with long-rank analysis were used for survival.
Results: : From 478 patients (74% w, 44±23 y at diagnosis, 10±8 y of follow-up, 28% PM, 22% DM, 19,9% OM, 19,2% JuvM, 8,4% CAM, 1,3% IBM, 1,3% NM), 98 (21%) presented cardiac involvement: 53 arrhythmia, 25 myocarditis and 30 pericarditis. Patients with CI were older at diagnosis (55 vs 40 y, p<.0001), and presented more frequently DM (p=.003). CI as a whole was associated with older age (p=.003), cardiovascular disease (CVD) (p<.0001), pulmonary hypertension (PH) (p=.005), and dysphagia (p=.016), less presence of calcinosis (p=.004), higher erythrocyte sedimentation rate (ESR) (p=.003). Arrhythmia was associated with older age (p=.008), CVD (p<.0001) and less calcinosis. Myocarditis was associated with pulmonary hypertension (p=.03), arterial hypertension (p=.008) and dysphagia (.001). Pericarditis was associated with CVD (p=.002), Raynaud (p=.008) and cancer (p=.03). Median survival was 16±3 and 35±10 years for patients with or without CI (p<.0001). Seven patients were lost to follow up and 47 (52%) died, mainly from CV event (32%), infection (28%), cancer (17%) and interstitial lung disease (4%).
Conclusion: Cardiac involvement appears in 20% patients with inflammatory myopathies and confers bad prognosis, with elevated mortality. Arrhythmia is associated with older age and cardiovascular disease, suggesting that other factors could influence survival, but myocarditis is associated with dysphagia, with could suggest the presence of a more severe disease.
To cite this abstract in AMA style:molina R, Joven BE, Nuño L, López Longo FJ, GARCIA DE YEBENES Y PROUS MJ, Maldonado V, Larena C, Llorente I, Tomero E, Barbadillo C, Garcia De La Peña P, Ruiz Gutiérrez L, Lopez-Robledillo JC, Moruno Cruz H, Pérez Gómez A, Cobo-Ibáñez T, Almodóvar González R, Lojo L, Carreira P. Cardiac Involvement in Inflammatory Myopathies: A Report from the Remicam Registry [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/cardiac-involvement-in-inflammatory-myopathies-a-report-from-the-remicam-registry/. Accessed October 31, 2020.
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