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Asymptomatic giant coronary aneurysm in an adolescent with Behcet's syndrome

DOI: 10.1186/1546-0096-10-2

Keywords: Behcet's syndrome, vasculitis, coronary aneurysm

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Abstract:

De-identified data were collected retrospectively in case report format. Although our sixteen year old female with Behcet's vasculitis had resolution of many arterial aneurysms, she had persistent venous thrombosis of large vessels, as well as persistent, giant arterial aneurysms requiring intra-arterial coiling of a lumbar artery and coronary bypass grafting despite intensive immunosuppression including glucocorticoids, cyclophosphamide, infliximab, methotrexate, azathioprine and intravenous immunoglobulin.Vascular manifestations may be seen in Behcet's syndrome, including asymptomatic coronary aneurysm, which may be refractory to immunosuppression and ultimately require surgical intervention. Increased awareness is essential for prompt diagnosis and management.Behcet's syndrome (BS) was initially described as a triad of symptoms including uveitis with oral and genital ulcerations [1]. It has since been expanded to include a constellation of recurring clinical manifestations with variable mucocutaneous, neurologic, ophthalmologic, cardiovascular, gastrointestinal, constitutional and musculoskeletal manifestations. Cardiovascular manifestations are uncommon; vascular lesions may affect both venous and arterial systems, and can occur in both large and small arteries. Aneurysms are a rare, potentially life-threatening manifestation of BS, with coronary aneurysm rarely reported.We present the case of a teenage girl who developed medically refractory and life threatening Behcet's vasculitis, involving both small and large venous and arterial systems including a gigantic coronary aneurysm.The patient has given consent for the publication of her case report. A 16 year old female from the Dominican Republic presented with a three month history of malaise, fever, and anorexia and a ten pound weight loss. She also reported recurrent painful oral and genital ulcerations, as well as tender erythematous nodules over her lower legs, suggestive of erythema nodosum. Her laboratory

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