Department of Pediatrics, New York University Medical Center, 160 E, 32nd Street, New York, NY 10016, USA.
Pediatr Rheumatol Online J. 2012 Jan 6;10:2. doi: 10.1186/1546-0096-10-2.
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.
白塞病是一种特发性多器官综合征，可在儿童期发病。血管受累并不常见，很少有报道冠状动脉瘤形成。我们报告了一例青少年女孩患有难治性危及生命的白塞病血管炎，涉及小静脉和大静脉以及包括巨大冠状动脉瘤在内的动脉系统。
以病例报告的形式回顾性收集了去识别数据。尽管我们 16 岁的白塞病血管炎女性患者许多动脉动脉瘤已消退，但她仍有大血管持续性静脉血栓形成，以及持续存在的巨大动脉动脉瘤，需要进行经皮腔内血管成形术治疗，包括腰椎动脉和冠状动脉旁路移植术，尽管进行了强化免疫抑制治疗，包括糖皮质激素、环磷酰胺、英夫利昔单抗、甲氨蝶呤、硫唑嘌呤和静脉注射免疫球蛋白。
血管表现可能见于白塞病综合征，包括无症状性冠状动脉瘤，可能对抗免疫抑制治疗有抵抗，最终需要手术干预。提高认识对于及时诊断和治疗至关重要。
