中国呼吸与危重监护杂志

中国呼吸与危重监护杂志

大动脉炎肺动脉受累患者的临床特征研究

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目的探讨大动脉炎肺动脉受累患者的临床特点,以提高对该病的认识。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 月间收治大动脉炎肺动脉受累患者的临床资料。结果10 年间安贞医院共收治大动脉炎患者 233 例,肺动脉受累患者 17 例,占同期所有大动脉炎患者的 7.3%。其中,男 3 例,女 14 例,男女比例 1∶4.67。确诊年龄 16~59 岁,平均(40±13)岁。临床表现最常见的症状为活动后气促(15 例,88.2%),最主要的体征为双上肢收缩压差>10 mm Hg(9 例,52.9%),其次为脉搏减弱或无脉(6 例,35.3%)以及受累血管局部可闻及血管杂音(6 例,35.3%)。超声心动图检查发现 13 例患者有肺动脉高压,肺动脉造影显示多累及肺叶、段动脉,单侧多于双侧,右侧多于左侧。13 例患者接受糖皮质激素及免疫抑制剂治疗,11 例抗凝或抗血小板治疗;5 例行降肺动脉压的靶向治疗。结论大动脉炎肺动脉受累患者的临床表现无特异性,误诊率高。应提高大动脉炎合并肺动脉受累的早期诊断意识,及早干预,改善患者预后。

ObjectiveTo enhance the understanding of Takayasu’s arteritis (TA) with pulmonary vascular involvement through the analyses on clinical features.MethodsA retrospective study was conducted to analyze the clinical records of patients diagnosed as TA with pulmonary vascular involvement admitted to Beijing Anzhen Hospital from January 2007 to August 2017.ResultsIn recent 10 years, there were 233 patients diagnosed as TA in Beijing Anzhen Hospital, 17 of them were involved with pulmonary arterial, which accounted for 7.3%. Fourteen patients were females, 3 patients were males. The age ranged from 16 to 59 years with an average of (40±13) years. Among the 17 patients, dyspnea (15 cases, 88.2%) was the most common symptom, and unequal blood pressure of upper limbs (9 cases, 52.9%) was the main sign. Thirteen patients had pulmonary hypertension by echocardiographic examination. Angiographic data showed that multi-lobular and multi-segmental pulmonary branches were predominantly affected. Unilateral involvement of pulmonary artery was more common than bilateral involvement, while the right pulmonary arteries were more often affected than the left. Thirteen patients received hormone and immunosuppressive therapy, 11 patients received anticoagulation or antiplatelet therapy, and 5 patients received targeted treatment of pulmonary artery pressure.ConclusionsThe clinical manifestation of TA is unspecific and misdiagnosis rate is relatively high. Improving the understanding of TA is necessary so as to reduce the misdiagnosis rate at an early stage and ameliorate the prognosis.

关键词: 大动脉炎; 肺动脉高压; 临床特点

Key words: Takayasu’s arteritis; Pulmonary arterial hypertension; Clinical features

引用本文: 肖瑶, 杨京华, 黄小勇, 张颖, 吴春婷. 大动脉炎肺动脉受累患者的临床特征研究. 中国呼吸与危重监护杂志, 2018, 17(4): 364-368. doi: 10.7507/1671-6205.201803040 复制

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