白塞综合征20例临床特征及心血管影像学分析

Analysis of cardiovascular imaging and clinical features in 20 cases of Behcet’s syndrome

回顾性分析2018年1月—2020年8月首都医科大学附属北京安贞医院风湿免疫科治疗的白塞综合征患者20例的临床及影像学检查资料。20例患者中,男4例,女16例,平均年龄41.2岁,平均病程17.0年。超声、CT及MR检查发现心血管系统受累13例,主要表现为主动脉瓣脱垂并重度关闭不全,主动脉瘤、主动脉假性动脉瘤、主动脉溃疡、主动脉分支血管狭窄或闭塞等;肺栓塞2例,下肢静脉血栓1例。全部患者均接受激素和/或免疫抑制剂治疗。3例行主动脉瓣置换术,术后随访6个月,发生瓣周漏1例并行二次Bentall术治疗,主动脉瓣下假性动脉瘤1例,无异常1例。2例行腹主动脉腔内隔离术治疗,术后随访1年,发现支架周围脓肿1例,支架远端假性动脉瘤1例。结果提示白塞综合征累及心血管系统时表现多样,早期诊断及手术治疗困难。临床联合应用多种无创心血管影像学检查手段,可为白塞综合征患者心血管系统受累的诊断、治疗、随访及预后评估提供全面的心血管病变影像学信息。

The clinical and imaging data of 20 patients with Behcet’s syndrome admitted to Beijing Anzhen Hospital,Capital Medical University from January 2018 to August 2020 were collected for retrospective analysis.Among the 20 patients,16 were female(80%),with an average age of 41.2 years and an average clinical course of 17.0 years.13 cases were found to be involved in the cardiovascular system through Ultrasound,CT and MR imaging examinations of the cardiovascular system.The main manifestations were aortic valve prolapse and severe regurgitation,aortic aneurysm,aortic pseudoaneurysm,aortic ulcer,aortic branch vascular stenosis,occlusion,etc.;another 2 cases of pulmonary embolism,1 case of lower limb venous thrombosis.All patients received hormone and/or immunosuppressive therapy.3 cases underwent aortic valve replacement,and the imaging examination for half a year after the operation revealed 1 case of paravalvular leak and performed second Bentall operation,1 case of subvalvular pseudoaneurysm.Two cases underwent endovascular isolation of the abdominal aorta,and 1 case of peristent abscess and 1 case of pseudoaneurysm at the distal end of the stent were found in imaging examination during the 1-year follow-up.The results indicate that Behcet’s syndrome has various manifestations when it involves the cardiovascular system,and early diagnosis and surgical treatment are difficult.The combined clinical application of a variety of non-invasive cardiovascular imaging examination methods can provide comprehensive imaging information for the diagnosis,treatment,follow-up and prognosis evaluation of cardiovascular system involvement in patients with Behcet’s syndrome.