2018 年第 4 期 第 13 卷

多发性大动脉炎累及肺动脉的临床特点及误诊分析

Clinical features and misdiagnosis reasons of Takayasu′s arteritis with pulmonary artery involvement

作者：肖瑶杨京华张颖吴春婷张文美

英文作者：

单位：100029首都医科大学附属北京安贞医院呼吸与危重症科

英文单位：

关键词：多发性大动脉炎；肺动脉；误诊

英文关键词：

• 摘要：

• 目的    探讨多发性大动脉炎（TA）累及肺动脉的临床特点，分析常见误诊原因，以提高对该疾病的认识。方法    回顾性分析首都医科大学附属北京安贞医院2007年1月至2017年8月收治的17例TA累及肺动脉患者的临床资料，包括发病经过和误诊情况、临床症状和体格检查、实验室及影像学检查、治疗及转归等。结果    10年间TA累及肺动脉患者占同期所有TA患者的7.3%（17/233）。患者大多以活动后气短［11例（64.7%）］为首发症状就诊。17例患者中发病初期有15例被误诊，误诊率高达88.2%，误诊时间1个月～20年不等。常见误诊疾病为肺栓塞(7例)、心脏瓣膜病(5例)、肺炎(4例)、肺结核（3例）和肺动脉缺如（1例）。所有误诊病例均经过相应的抗凝、抗感染、抗结核等治疗，疗效不佳。其中2例误诊为慢性血栓栓塞性肺动脉高压，收入心脏外科预行肺动脉血栓内膜剥脱术；1例在术前确诊TA，内科保守治疗至今7年病情稳定；另1例在术中诊断TA，左肺动脉闭塞，右肺动脉分支附壁血栓至重度狭窄，行右肺动脉血栓内膜剥脱术，但术后由于残余肺动脉高压，继发肺动脉高压危象，多脏器功能衰竭，于术后11 d死亡。结论    TA累及肺动脉临床无特异性表现，误诊率高，肺栓塞为常见误诊原因，应提高TA合并肺动脉受累的早期诊断意识，降低误诊率，改善患者预后。

• Objective    To analyze clinical features and misdiagnosis reasons of Takayasu′s arteritis(TA) with pulmonary artery involvement. Methods    Clinical records of 17 patients diagnosed of TA with pulmonary artery involvement in Beijing Anzhen Hospital, Capital Medical University were retrospectively analyzed from January 2007 to August 2017. Disease process and misdiagnosis reasons, symptoms and manifestations, laboratory and imaging results, treatments and prognosis were analyzed. Results   TA with pulmonary artery involvement accounted for 7.3%（17/233） of all TA patients in 10 years. The initial symptom was post-activity shortness of breath in most patients［11 cases（64.7%）］. The misdiagnosis rate was 88.2%(15/17); the misdiagnosis time ranged from 1 month to 20 years. Common misdiagnosed diseases included pulmonary embolism(7 cases), valvular heart disease(5 cases), pneumonia(4 cases), pulmonary tuberculosis(3 cases) and absent pulmonary artery(1 case). All misdiagnosed cases were treated by anticoagulant, anti-infection and anti-tuberculosis therapies with no significant effect. Two patients were misdiagnosed of chronic thromboembolic pulmonary hypertension; 1 of them was diagnosed of TA before operation and had conservative medical treatments for 7 years; another patient was diagnosed of TA during pulmonary endarterectomy and died 11 d after operation due to pulmonary hypertensive crisis. Conclusion    sTA with pulmonary artery involvement has unspecific clinical manifestations and high misdiagnosis rate. Pulmonary embolism is a common misdiagnose reason.