血管型白塞综合征

Vasculo-Behcet' s syndrome:a case report

报告1例以多发性假性动脉瘤为主要表现的反复误诊误治的血管型白塞综合征。患者男,33岁。因全身反复出现搏动性包块9个月就诊。多次CT血管造影检查示假性动脉瘤,并先后在血管外科行3次手术治疗,但第3次手术后1个月右腋下再次发生假性动脉瘤。追问病史患者近10年来口腔和外生殖器反复发生溃疡。体格检查:除右腋下一鸡蛋大的搏动性包块外,无其他阳性体征。诊断:血管型白塞综合征。患者经口服泼尼松45 mg/d和每2周静脉滴注环磷酰胺1 200 mg冲击治疗,1个月后右腋下包块变硬、缩小,随后调整治疗方案为硫唑嘌呤2 mg/(kg·d)和泼尼松逐渐减量口服。6个月后右腋下包块消失,随访1年,无新的血管损害发生。

A case of vasculo-Behcet＇s syndrome with muhiple pseudo-aneurysms in large arteries is reported. A-33-yearold man was admitted with multiple pulsating subcutaneous mass for 9 months. Every time computed tomography angiogram revealed pseudo-aneurysm. The patient undergone three surgeries, but one month after his third operation, he developed a pulsatile swelling in right axilla again. Then a detailed review of history revealed recurrent oral and genital aphthous ulcers of ten-year duration. Physical examinations showed a pulsatile swelling in right axilla. Other systemic examinations were unremarkable. A diagnosis of vaseulo-Behcet＇s disease was made. Then oral prednisone 45 mg daily and pulses of intravenous cyclophosphamide 1 200 mg every two weeks was given. One month later, the mass was hardened and shrinking. Then the patient received a regimen of azathioprine （2 mg/kg.d） and prednisone, which were tapered in small steps. Six months later, the mass was resolution. Subsequently there has been no episode of any new vascular lesion at the one-year follow up.