A coronary artery aneurysm revealing a Behçet’s disease: a case report

Behçet’s disease (BD) is a multisystemic chronic vasculitis characterized by its clinical polymorphism. It concerns mainly young men and generally appears between the third and the fourth decades. Cardiac involvement in Behçet’s disease is rare but represents a major prognostic factor. We report the case of a young man admitted in our department for the management of an acute coronary syndrome revealing a Behçet’s disease. Coronary angiography had shown a giant thrombosed aneurysm of the left coronary artery. Surgical treatment was successfully performed and the patient had a left anterior descending coronary artery bypass using the left internal mammary artery graft.


Introduction
First described in 1937, Behçet's disease (BD) is defined as a chronic multisystemic inflammatory condition affecting small, medium and large-caliber vessels [1,2]. Its prevalence varies widely from one region to another and is particularly high in the Middle and Far East [3]. Genetic, environmental or autoimmune factors have been implicated in its etiopathogenesis, however, the exact etiology remains unclear [2]. The disease usually appears during the 3 rd or the 4 th decade with a peak frequency around the age of 30 [1]. Both men and women could be affected, but there is a slight male predominance [4]. Cardiac involvement in BD is uncommon, however, it is clearly associated with a poor prognosis [2]. We report the case of a young man admitted in our department for acute coronary syndrome secondary to a thrombosed aneurysm of the left anterior descending coronary artery and revealing an angiobehçet.

Patient and observation
A 32-year-old man, with no cardiovascular risk factors, was admitted in our department for typical chest pain that had been exaggerated in the last week. In his past medical history, he had a left hip synovitis treated with corticosteroids and a recurrent oral aphthosis.

Discussion
Cardiac involvement is rare but severe during BD. Its prevalence varies from 0.36 to 19.5 per 100,000 inhabitants depending on the clinical series [1,5]. All three layers of the cardiac wall can be affected.
Thus, cardiac manifestations may include, pericarditis (20 to 40%), myocarditis (20%), endocarditis, endomyocardial fibrosis or myocardial infarction [6]. The coronary arteries are rarely involved (prevalence of 0.5%) by stenotic lesions, arteritis or especially aneurysms formation [4]. Behçet disorder is rarely inaugurated by coronary artery disease. In fact, most cases reported in the literature were already diagnosed and treated for this condition [2]. Typical clinical picture is represented by acute coronary syndrome and the first case was reported by Schiff et al. in 1982 [7]. Several mechanisms have been described in the literature: coronary occlusions secondary to vasculitis, the formation of thrombus in situ, compression of the vessels by an aneurysm or dilated valsalva sinus or less frequently an impairment of the microvascular function [2,8]. The modalities for the management of coronary artery disease during BD are based on the decision of a multidisciplinary team including the internist, the cardiologist and the cardiovascular surgeon. In general, in addition to standard antiischematic therapy, the combination of corticosteroids with immunosuppressive drugs is highly recommended [9].
Complete remission can be achieved by medical treatment alone and some aneurysms may even completely regress. Nevertheless, surgical or endovascular approaches are deemed necessary in most circumstances. Surgery is recommended for giant aneurysms (size greater than 2 cm), with fast progression and high risk of rupture [3].
In other cases, surgical or interventional management can be discussed [4,10,11]. These two techniques may, however, carry more risk of disease progression which is inherent to their pathergylike effects [12]. Several measures can reduce this risk, such as optimal control of inflammation in the preoperative period, careful examination of arterial or venous grafts before their implantation, and avoiding excessive manipulation of the aorta [2,12].

Conclusion
Coronary involvement during BD is rare but serious. Angiographically, the most commonly found coronary lesions are aneurysms for which treatment could be surgical or endovascular. Due to the unpredictable evolution of this condition and insufficient data concerning the medium and long-term results of both surgical and interventional techniques, long-term follow-up is required.