ANCA相关性血管炎并发气胸患者的临床特点

Clinical feature of patients with anti-neutrophil cytoplasmic antibody associated vasculitis combined with pneumothorax

目的 分析ANCA相关性血管炎(anti-neutrophil cytoplasmic antibody associated vasculitis,AAV)并发气胸患者的临床特点及结局.方法 回顾性分析北京协和医院2007年1月1日至2017年12月31日住院AAV并发气胸患者的临床资料,同期住院无气胸的33例AAV患者为对照.结果 11例AAV患者中5例为肉芽肿性多血管炎(granulomatosis with polyangiitis,GPA),5例为显微镜下多血管炎(microscopic polyangiitis,MPA),1例为AAV;9例为单纯气胸,1例为单侧包裹性液气胸,1例为脓气胸.气胸组肺部空洞病变、 肺部感染、 呼吸衰竭和菌血症的发生率和死亡率明显高于对照组(P<0.05).死亡组呼吸衰竭的发生率明显高于存活组(P<0.05).结论 AAV并发气胸与原发病累及肺和胸膜、 原发病活动和肺部感染相关.AAV并发气胸患者的气胸较难控制、 死亡率高,尤其是并发呼吸衰竭时.

Objective To investigate the clinical features and outcomes of anti-neutrophil cytoplasmic antibody associated vasculitis(AAV)patients combined with pneumothorax.Methods Retrospectively collected 11 inpatients in Peking Union Medical College Hospital from January 1,2007 to December 31,2017,who had been diagnosed as AAV combined with pneumothorax.At the same time,we manually selected 33 AAV patients without pneumothorax as controls.Results Among these 11 patients,5 were granulomatosis with polyangiitis(GPA),5 microscopic polyangiitis(MPA),and 1 unclassified AAV.There were 9 patients with simple pneumothorax,and individual unilateral encapsulated liquid pneumothorax and pyopneumothorax.The incidence of pulmonary cavity,pulmonary infection,respiratory failure,bacteremia,and mortality rate in patients with AAV with pneumothorax was higher than those in the control group(P<0.05).The incidence of respiratory failure in the death group was significantly higher than the survival group(P<0.05).Conclusions AAV with pneumothorax is rare.The reason of pneumothorax in AAV patients may be associated with primary lung and pleural involvement,primary disease activity,and pulmonary infection.Pneumotho-rax in AAV patients was difficult to control and the patients had a high mortality,especially in those with respiratory failure.