Cardiac Involvement in Inflammatory Myopathies: A Report from the Remicam Registry

rina molina¹, Beatriz E. Joven², Laura Nuño³, Francisco Javier López Longo⁴, MARIA JESUS GARCIA DE YEBENES Y PROUS⁵, VAlentina Maldonado⁶, Carmen Larena⁷, Irene Llorente⁸, Eva Tomero⁹, Carmen Barbadillo¹⁰, Paloma Garcia De La Peña¹¹, Lucía Ruiz Gutiérrez¹², Juan Carlos Lopez-Robledillo¹³, Henry Moruno Cruz¹², Ana Pérez Gómez¹², Tatiana Cobo-Ibáñez¹⁴, Raquel Almodóvar González¹⁵, Leticia Lojo¹⁶ and Patricia Carreira¹⁷, ¹MADRID, Hospital 12 de Octubre, MADRID, Spain, ²Rheumatology, Hospital 12 de Octubre, Madrid, Spain, ³Servicio de Reumatologia, Hospital Universitario La Paz, Madrid, Spain, ⁴Rheumatology, Hospital Gregorio Marañón, Madrid, Spain, ⁵INSTITUTO DE SALUD MUSCULOESQUELÉTICA, MADRID, Spain, ⁶Hospital Ramón y Cajal, Madrid, Spain, ⁷Hospital Gregorio Marañón, Madrid, Spain, ⁸Rheumatology, H.U. La Princesa, Madrid, Spain, ⁹Hospital La Princesa. Madrid., Madrid, Spain, ¹⁰Hospital Universitario Puerta de Hierro, Madrid, Spain, ¹¹Rheumatology, Hospital Madrid Norte Sanchinarro, Madrid, Spain, ¹²University Hospital Príncipe de Asturias, Immune System Diseases, Rheumatology department, Alcalá de Henares, Madrid, Spain, ¹³Hospital Niño Jesus, Madrid, Spain, ¹⁴Hospital Universitario Reina Sofía, Universidad Europea de Madrid, Madrid, Spain, ¹⁵Rheumatology Unit, Hospital Universitario Fundación Alcorcón, Madrid, Spain, ¹⁶Rheumatology, Hospital Universitario La Paz, Spain, Spain, ¹⁷Department of Rheumatology, Hospital Universitario 12 de Octubre, Madrid, Spain

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: Myocardial involvement, myopathy and myositis

Session Information

Date: Tuesday, November 15, 2016

Title: Muscle Biology, Myositis and Myopathies - Poster II: Clinical

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.

Disclosure: R. molina, None; B. E. Joven, None; L. Nuño, None; F. J. López Longo, None; M. J. GARCIA DE YEBENES Y PROUS, None; V. Maldonado, None; C. Larena, None; I. Llorente, None; E. Tomero, None; C. Barbadillo, None; P. Garcia De La Peña, FUNDACION ESPAÑOLA DE REUMATOLOGIA, 2; L. Ruiz Gutiérrez, None; J. C. Lopez-Robledillo, None; H. Moruno Cruz, None; A. Pérez Gómez, None; T. Cobo-Ibáñez, None; R. Almodóvar González, None; L. Lojo, None; P. Carreira, None.

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 .

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