Ab0798 prevalence and clinical spectrum of cardiac involvement in behçet’s syndrome: a single center retrospective study

Background Behçet’s Syndrome (BS) is a systemic vasculitis, which main clinical features include mucocutaneous manifestations, pan-uveitis, non-deforming arthritis, thrombosis, central nervous system (CNS) and gastrointestinal (GI) involvement [1] . Despite being increasingly recognized as part of the clinical spectrum in BS [2,3,4] , cardiac involvement has not been systematically described. Objectives To investigate the prevalence and clinical spectrum of cardiac manifestation in a cohort of BS patients. Methods Three hundred and twelve patients were retrospectively studied. All patients fulfilled the International Classification Criteria for BS [5] . Demographic, clinical, therapeutic features, and specific data on cardiac involvement were collected. Results Cardiac involvement was observed in 46 out of 312 patients (13.2%). The mean age at cardiac involvement diagnosis was 43 years (SD ± 13.2). Mean BS duration before cardiac manifestation onset was 5.35 years (SD ± 6.86). Female to male ratio was 1.42. Among 46 patients with cardiac involvement, 37 (80.4%) displayed a single manifestation, while 9 (19.5%) showed two or more cardiac events. BS related cardiac lesions included arrhythmias (n=12; 26%), pericarditis (n=12; 26%), ischemic heart disease (n=7; 15%), acute myocarditis (n=5; 10.8%), valvular abnormalities (n=8; 17.3%), and pulmonary hypertension (n=2; 4.3%) (Figure 1 ). Thirteen patients (28.2%) had cardiac abnormalities classified as “other”, which included patent foramen ovale, Takotsubo syndrome, cardiac amyloidosis, and aortic root aneurysm (Figure 1 ). After the first event, remission of cardiac manifestations was achieved by all patients. Cardiac relapse occurred in 9 patients (19.5%), due to recurrent pericarditis (n=7), myocarditis (n=1), and arrhythmic manifestation (n=1). Overall clinical manifestations of BS are shown in Table 1 . Muco-cutaneous manifestations were present in almost all patients, specifically oral aftosis was present in 45/46 (98%), genital aftosis in 26/46 (56.5), and skin manifestations in 35/46 (76%). A significant proportion of patients displayed vascular involvement (n=20; 43,4%), CNS involvement (n=20; 43.4%), and GI involvement (n=32; 69.5%). At the time of cardiac events 21/46 (45.6%) patients were receiving oral corticosteroids, 16/46 (34.7%) were receiving colchicine, 13/46 (28.2%) were receiving a traditional DMARD, while 19/46 (41.3%) were receiving a biologic DMARD (Table 1 ). Conclusion Our study indicates that cardiac events are a fairly common manifestation in BS patients, pointing out the need for a routine screening of such involvement. As previously described [2] , our study suggests the association between cardiac abnormalities and vascular involvement in BS. Furthermore, we found a significant proportion of patients presenting with other major organ manifestations, such as CNS and GI involvement, suggesting that cardiac involvement may reflect a more severe and systemic disease course. References [1]Bettiol Rheumatology (Oxford) 2020 [2]Geri Medicine (Baltimore) 2012 [3]Chen Clin Rheumatol 2019 [4]Kechida Adv Rheumatol 2018 [5]International Team for the Revision of the International Criteria for Behçet’s Disease J Eur Acad Dermatol Venereol 2014 Figure 1. Spectrum of cardiac lesions in a monocentric cohort of BS patients Table 1. Demographic and clinical features of BS patients with cardiac involvement BS patients (46) Female N (%) 27 (58.6%) Age (mean ± SD) 43 (± 13.2) Cardiovascular risk factors N (% ) Smoking habit 7 (15.2%) Dyslipidemia 11 (24%) Hypertension 10 (21.7%) Diabetes 2 (4.3%) Clinical manifestations N (% ) Oral aftosis 45 (98%) Genital aftosis 26 (56.5%) Ocular involvement 12 (26%) Skin manifestations 35 (76%) Positive pathergy test 11 (24%) Vascular involvement 20 (43.4%) CNS involvement 20 (43.4%) GI involvement 32 (69.5%) Articular involvement 33 (71.7%) HLA-B51 positivity 30 (65.2%) Treatment N (% ) Corticosteroids 21 (45.6%) Colchicine 16 (34.7%) Traditional DMARDs 13 (28.2%) Biologic DMARDs 19 (41.3%) Acknowledgements: NIL. Disclosure of Interests None Declared.