[Clinical analysis of Behçet disease associated with pulmonary vascular disease].

Objective: To investigate the clinical features, therapy and prognosis of Behcet's disease(BD) complicated with pulmonary vascular diseases (PVD). Methods: The clinical manifestation, hemodynamics, pulmonary artery angiographic findings, therapy and prognosis of PVD in BD in Shanghai Pulmonary Hospital from January 2009 to August 2016 were analyzed retrospectively. Results: Seven patients with average of (37±20) years were included. The ratio of male vs. female was 6∶1. Vascular involvement included pulmonary hypertension (PH) (4/7), pulmonary embolism (PE) (3/7) and pulmonary artery aneurysms (PAA) (3/7). All patients received immunosuppressive agents and all PH patients had therapy of PH target drugs. Three out of 4 PE patients underwent anticoagulant therapy. Conclusion: PVD is a rare complication of BD. The common clinical manifestations are PH, PE and PAA. Management of immunosuppressive agents may improve the prognosis and should be recommended.目的： 总结白塞病合并肺血管疾病(PVD)患者的临床特点和诊治策略。 方法： 回顾性总结同济大学附属上海市肺科医院2009年1月至2016年6月7例白塞病合并PVD住院患者的临床资料，其中男6例，女1例，年龄20～69岁，平均发病年龄(37±20)岁。分析本组患者的血流动力学、肺部影像学特点、治疗及预后。 结果： 白塞病合并PVD患者临床主要表现为肺动脉高压(4/7)、肺栓塞(3/7)和肺动脉瘤(3/7)。肺动脉造影主要表现为肺动脉狭窄闭塞性病变或瘤样扩张性病变，部分可伴有瘤内血栓形成。7例患者均接受糖皮质激素联合免疫抑制剂治疗，1例肺动脉高压患者接受靶向药物治疗，2例肺栓塞患者接受抗凝治疗。7例患者平均随访时间为(83±40)个月，均存活。 结论： 白塞病可罕见累及肺血管系统，以肺动脉高压、肺栓塞和(或)肺血管瘤为主要表现。及时合理加强免疫抑制治疗，可能有助于改善患者的预后。.