Cardiac complication spectrum of Behçet disease in Tunisia: A 10-case series: Clinical and therapeutic approach

Abstract

Introduction:Cardiac involvement (CI), although rare, remains one of the most severe complications of Behçet Disease (BD).Objectiveto investigate the frequency and spectrum of cardiac involvement in BD and to assess the clinical and imaging features, treatment, and outcomes.MethodsWe retrospectively retrieved the medical records of patients with CI among 220 BD patients admitted to the internal medicine department between 2006 and 2016 who fulfilled the International Study Group diagnostic criteria for the classification of BD.ResultsTen patients were eligible for the study with a sex ratio (male/female) of 8/10. Three cases had 2 isolated episodes of cardiac BD. The mean age when diagnosing the first cardiac episode was 37,3-year-old. The different types of CI were: coronary artery disease (5/10), intracardiac thrombus (4/10) myocarditis (1/10), pericarditis (1/10), myocardial fibrosis (1/10). Vascular involvement was associated to CI in 5 cases (50%). The main symptoms were chest pain (80%), fever (60%), dyspnea (50%), cough (10%) and hemoptysis (10%). The laboratory tests revealed increased inflammatory markers in 5 patients. Medical treatment was based on colchicine and corticosteroid in all patients (100%), anticoagulants in 8 patients (80%), Cyclophosphamide followed by Azathioprine in 9 patients (90%), Azathioprine monotherapy was started in one patient (10%). Antiplatelet and anti-ischemic therapy in 5 patients who had coronary artery disease. The evolution was favorable in 9 cases and marked by severe heart failure and the death of one patient.ConclusionCI despite its rarity, remains an important feature of BD due to the increased risk of mortality and morbidity. Thus, early screening and detection are paramount. Also, imaging has been of great contribution to diagnose such complications and hence indicate the adequate treatment, including immunosuppressant agents.