Premature coronary disease, in-stent restenosis and vascular complications in a young man with Behçet syndrome.

A young man with a history of early-onset coronary disease presented with an ST-elevation myocardial infarction at the age of 38. He subsequently had recurrent in-stent restenosis requiring repeating interventions and ultimately bypass surgery. After 4 years, he presents with systemic symptoms, new skin lesions and a femoral artery pseudoaneurysm. He is diagnosed with Behçet syndrome, a rare systemic vasculitis characterised by the triad of oral aphthous ulcers, genital ulcers and ocular involvement. Behçet is not associated with premature coronary disease but can have a variety of cardiac complications. Additionally, pathergy, an exaggerated inflammatory response to local injury, is characteristic. We hypothesise that in retrospect, subclinical inflammation and a vascular pathergy likely predisposed him to his cardiac and vascular complications. Here, we review risk factors and presentation of premature coronary artery disease and review the literature on the cardiovascular complications of Behçet syndrome.