Diffuse coronary artery aneurysms involving three major vessels in a patient with Behçet’s disease

Behçet’s disease (BD) is a multiorgan vasculitis of unknown origin, with characteristic skin lesions, recurrent uveitis and neurological manifestations [1–3]. Here we present a case of diffuse coronary artery aneurysms involving three major vessels and its appropriate medical treatment.
A 45-year-old man visited our cardiology outpatient clinic with a 2-month history of progressive exertional chest pain (Canadian Cardiovascular Society Class III). The patient had a history of BD, which had been diagnosed 15 years ago, and he was not routinely followed up in our rheumatology clinic due to social reasons. He had no cardiac risk factor. On physical examination he was afebrile, pulse rate was 85 per minute and blood pressure was 130/80 mm Hg. The electrocardiogram revealed normal sinus rhythm. Laboratory tests showed normal high-sensitivity C-reactive protein and negative troponin-T level. Transthoracic echocardiography revealed no abnormality. Exercise stress test showed ST segment depression in leads I, aVL and V4–6, and a coronary angiogram was performed. It demonstrated diffuse fusiform aneurysms located on the left anterior descending (LAD), circumflex (Cx) and right coronary (RCA) arteries with extensive coronary slow flow (Figure 1 A–C). Maximal coronary aneurysm diameter was measured as 6.4 mm for the LAD, 6.8 mm for the Cx and 8.3 mm for the RCA in coronary computed tomography (CT) angiography (Figure 1 D). The patient was treated with anti-ischemic agents, including acetylsalicylic acid, a -blocker, a statin, an ACE inhibitor and oral nitrates. Thereafter, rheumatology consultation was requested. Intravenous cyclophosphamide 1 g/month and methylprednisolone 1 mg/kg/day (with gradual dose tapering) were initiated as the immunosuppressive (IS) treatment. During the hospital course the patient did not complain of any chest pain, and he was discharged with the same anti-ischemic and IS treatment. One month after discharge, his functional class had decreased to CCS Class I–II. As the patient’s symptoms regressed with proper medical treatment, surgical or endovascular interventions were not considered. Three months after discharge, coronary CT angiography revealed no increase in the size of each aneurysm. The patient has been in the regular outpatient clinic follow-up for 1 year, without any increase in his symptoms.
Behcet’s disease is a chronic autoimmune vasculitis that may cause single or multiple coronary aneurysms. Patients may be...


View full text...