title: Spectrum of cardiac lesions in Behçet disease: a series of 52 patients and review of the literature.
abstract: Cardiac abnormalities in patients with Behçet disease (BD) include pericarditis, myocarditis, endocarditis with valvular regurgitation, intracardiac thrombosis, endomyocardial fibrosis, coronary arteritis with or without myocardial infarction, and aneurysms of the coronary arteries or sinus of Valsalva. Data regarding the clinical spectrum, prevalence, and outcome of cardiac lesions in BD are lacking. In this study, we report the main characteristics, treatment, and long-term outcomes of 52 patients with cardiac lesions from a cohort of 807 (6%) BD patients. Forty-five (86.5%) patients were male, with a mean (±SD) age at BD diagnosis of 29.3 ± 10.3 years.Cardiac involvement was the first feature of BD in 17 (32.7%) patients. Cardiac lesions included pericarditis (n = 20; 38.5%), endocarditis (mostly aortic insufficiency) (n = 14; 26.9%), intracardiac thrombosis (n = 10; 19.2%), myocardial infarction (n = 9; 17.3%), endomyocardial fibrosis (n = 4; 7.7%) and myocardial aneurysm (n = 1; 1.9%). Patients with cardiac involvement were more frequently male (86.5% vs. 64.9%; p < 0.01) and had more arterial (42.3% vs. 11.1%; p < 0.01) and venous lesions (59.6% vs. 35.8%; p < 0.01) compared to those without cardiac manifestations. Factors associated with complete remission of cardiac involvement were treatment regimens with oral anticoagulants, immunosuppressants, and colchicine. The 5-year survival rate was 83.6% and 95.8% (p = 0.03) in BD patients with and without cardiac involvement, respectively. After a median (Q1-Q3) follow-up of 3.0 (1.75-4.2) years, 8 patients had died, in 3 cases directly related to cardiac involvement.In conclusion, cardiac lesions affected 6% of our large cohort of BD patients. The prognosis of cardiac involvement in BD is poor and improves with oral anticoagulation, immunosuppressive therapy, and colchicine.
authors.affiliation: From Service de Médecine Interne 2 (GG, BW, DLTH, JCP, ZA, PC, DS) and Service de Cardiologie (RI), Groupe Hospitalier Pitié-Salpêtrière, Assistance Publique Hôpitaux de Paris, Paris; Laboratory I3 "Immunology, Immunopathology, Immunotherapy" (GG, PC, DS), UMR 7211 (CNRS/UPMC) INSERM U959, Hôpital Pitié-Salpêtrière, Université Pierre et Marie Curie, Paris 6; and Département de Biostatistique (MRR), Groupe Hospitalier Saint-Louis, Assistance Publique Hôpitaux de Paris, Paris, France.
authors.forename: Guillaume
authors.initials: G
authors.lastname: Geri
authors.forename: Bertrand
authors.initials: B
authors.lastname: Wechsler
authors.forename: Du Le
authors.initials: DL
authors.lastname: Thi Huong
authors.forename: Richard
authors.initials: R
authors.lastname: Isnard
authors.forename: Jean-Charles
authors.initials: JC
authors.lastname: Piette
authors.forename: Zahir
authors.initials: Z
authors.lastname: Amoura
authors.forename: Mathieu
authors.initials: M
authors.lastname: Resche-Rigon
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
mesh_terms: D000328:Adult; D000925:Anticoagulants / Q000627:therapeutic use; D001528:Behcet Syndrome / Q000150:complications / Q000188:drug therapy / Q000401:mortality / Q000473:pathology*; D015331:Cohort Studies; D003078:Colchicine / Q000627:therapeutic use; D003328:Coronary Thrombosis / Q000209:etiology / Q000473:pathology; D004696:Endocarditis / Q000209:etiology / Q000473:pathology; D004719:Endomyocardial Fibrosis / Q000209:etiology / Q000473:pathology; D005260:Female; D006322:Heart Aneurysm / Q000209:etiology / Q000473:pathology; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D008297:Male; D009203:Myocardial Infarction / Q000209:etiology / Q000473:pathology; D009206:Myocardium / Q000473:pathology*; D010493:Pericarditis / Q000209:etiology / Q000473:pathology; D012737:Sex Factors; D016896:Treatment Outcome
chemical_list: D000925:Anticoagulants; D007166:Immunosuppressive Agents; D003078:Colchicine