title: Myocarditis in Patients With Antisynthetase Syndrome: Prevalence, Presentation, and Outcomes.
abstract: Antisynthetase syndrome (aSS) corresponds to an overlapping inflammatory myopathy identified by various myositis-specific autoantibodies (directed against tRNA-synthetases). Myocardial involvement in this condition is poorly described.From a registry of 352 aSS patients, 12 cases of myocarditis were retrospectively identified on the basis of an unexplained increase in troponin T/I levels associated with either suggestive cardiac magnetic resonance imaging (MRI) findings, nonsignificant coronary artery abnormalities or positive endomyocardial biopsy.The prevalence of myocarditis in aSS is 3.4% and was not linked to any autoantibody specificity: anti-Jo1 (n = 8), anti-PL7 (n = 3), and anti-PL12 (n = 1). Myocarditis was a part of the first aSS manifestations in 42% of the cases and was asymptomatic (n = 2) or revealed by an acute (n = 4) or a subacute (n = 6) cardiac failure. It should be noted that myocarditis was always associated with an active myositis. When performed (n = 11), cardiac MRI revealed a late hypersignal in the T1-images in 73% of the cases (n = 8). Half of the patients required intensive care. Ten patients (83%) received dedicated cardiotropic drugs. Steroids and at least 1 immunosuppressive drug were given in all cases. After a median follow-up of 11 months (range 0-84) 9 (75%) patients recovered whereas 3 (25%) developed a chronic cardiac insufficiency. No patient died.The prevalence of myocarditis in aSS is similar to that of other inflammatory myopathies. Although the prognosis is relatively good, myocarditis is a severe condition and should be carefully considered as a possible manifestation in active aSS patients.
authors.affiliation: From the Service de Maladies Infectieuses et Maladies du Sang, Centre Hospitalier de Rochefort (CD); Service de Médecine Interne, Centre Hospitalier Universitaire de Fort de France (CD); Service de Rhumatologie, Centre National de Référence des Maladies Autoimmunes et Systémiques Rares, Hôpitaux Universitaires de Strasbourg (AM, JS); Service de Radiologie Vasculaire et Interventionnelle (PC); Département de Médecine Interne et d'Immunologie Clinique, Centre national de Référence des Maladies Neuromusculaires, DHU I2B, Hôpital Pitié-Salpêtrière, 47-83 Boulevard de l'Hôpital, Paris (NC, DS, OB, BH); Service de Médecine Interne - Centre national de Référence des Maladies Autoimmunes et Systémiques Rares, Hôpital Claude Huriez, Université de Lille, Lille (GL, EH); and Service de Médecine Interne, Hôpital Haut-Lévêque, Pessac, France (J-LP).
authors.forename: Céline
authors.initials: C
authors.lastname: Dieval
authors.forename: Christophe
authors.initials: C
authors.lastname: Deligny
authors.forename: Alain
authors.initials: A
authors.lastname: Meyer
authors.forename: Philippe
authors.initials: P
authors.lastname: Cluzel
authors.forename: Nicolas
authors.initials: N
authors.lastname: Champtiaux
authors.forename: Guillaume
authors.initials: G
authors.lastname: Lefevre
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
authors.forename: Jean
authors.initials: J
authors.lastname: Sibilia
authors.forename: Jean-Luc
authors.initials: JL
authors.lastname: Pellegrin
authors.forename: Eric
authors.initials: E
authors.lastname: Hachulla
authors.forename: Olivier
authors.initials: O
authors.lastname: Benveniste
authors.forename: Baptiste
authors.initials: B
authors.lastname: Hervier
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D005260:Female; D005602:France / Q000453:epidemiology; D006801:Humans; D008297:Male; D008875:Middle Aged; D009205:Myocarditis / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology*; D009220:Myositis / Q000150:complications* / Q000453:epidemiology; D015995:Prevalence; D012189:Retrospective Studies; D055815:Young Adult