Asymptomatic giant coronary aneurysm in an adolescent with Behcet's syndrome
1 Department of Pediatrics, New York University Medical Center, 160 E. 32nd Street, New York, NY 10016, USA
2 Department of Medicine, New York University Medical Center-Hospital for Joint Diseases, 246 e. 20th Street, New York, NY 10003, USA
3 Department of Radiology and Medicine, New York University Medical Center, 560 First Avenue, New York, NY 10016, USA
4 Department of Pediatrics, Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada
Pediatric Rheumatology 2012, 10:2 doi:10.1186/1546-0096-10-2Published: 6 January 2012
Behcet's is an idiopathic multi-organ syndrome, which may have onset during childhood. Vascular involvement is uncommon, with rarely reported coronary aneurysm formation. We present a case report of a teenager girl who developed recalcitrant life-threatening Behcet's vasculitis, involving both small and large venous and arterial systems including a giant coronary aneurysm.
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.