白塞病合并肺血管疾病临床特点分析

Clinical analysis of Behcet disease associated with pulmonary vascular disease

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。

To investigate the clinical features, therapy and prognosis of Behcet′s disease（BD） complicated with pulmonary vascular diseases （PVD）. MethodsThe 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. ResultsSeven 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. ConclusionPVD 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.