Large vessel vasculitis in Behçet’s disease

A 29-year-old female refugee from Afghanistan presented with gradual onset of exertional angina. She reported a history of percutaneous coronary intervention of her left anterior descending artery (LAD) at an Iranian clinic 8 months before with implantation of 3 drug eluting stents. Selective coronary angiography revealed an in-stent restenosis of the proximal LAD (Panel A, see Supplementary data online, Video S1Supplementary data online, Video S1), which was treated with a drug-eluting stent (Xience Xpedition 4.0/8mm) with a good angiographic result (see Supplementary data online, Video S2Supplementary data online, Video S2). Transthoracic and subsequent transoesophageal echocardiography exposed an 8 mm echogenic thickening of the posterior wall of the ascending aorta extending to the aortic valve (Panel B, see Supplementary data online, Videos S3 and S4Supplementary data online, Videos S3 and S4) and confirmed by computed tomography (Panel C). Fluorodeoxyglucose (FDG)-positron emission magnet resonance imaging revealed abnormal FDG uptake within the intraaortic wall in proximity to the left main coronary artery (Panel D) as well as the proximal left carotid artery. Our findings complemented by a patient history of oral and genital ulcerations, erythema nodosum, and arthralgia were suggestive for active large-vessel vasculitis in the setting of Behçet’s disease. Thus, immunsupressive treatment with prednisolone and cyclophosphamide was initiated and the patient was discharged in good condition.

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