摘要目的 探讨显微镜下多血管炎(microscopic polyangiitis,MPA)肺部病变特点.方法 回顾性分析2008年1月至2009年12月在北京协和医院伟院的50例MPA患者的临床资料,包括临床表现、实验室检查、超声心动图、肺功能、胸部CT及肾脏病理等.结果 男22例,女28例,年龄23～85岁,平均(66±12)岁.病程中存在肺部病变者46例(92%),常见症状有咳嗽(34/46,74%)、咳痰(30/46,65%)、呼吸困难(19/46,41%)和咯血(16/46,35%);14例(14/50,28%)以肺部病变为首发表现,其中5例影像学特点符合普通型问质性肺炎.抗中性粒细胞胞浆抗体(ANCA)中髓过氧化物酶(MPO)-ANCA阳性率96%(48/50),蛋白酶3(PR3)-ANCA阳性率6%(3/50).影像学表现为磨玻璃影者16例,肺间质纤维化16例,浸润影12例,胸腔积液7例.常见肺功能异常为弥散功能障碍(12/15)和限制性通气功能障碍(4/15).肺动脉高压13例,肺动脉收缩压平均为(48±8)mmHg(1 mm Hg=0.133 kPa).结论 MPA肺受累发生率高,约1/3患者以肺受累为首发表现;临床表现无特异性,磨玻璃影、肺间质纤维化、浸润影和胸腔积液为常见影像学表现;激素和免疫抑制剂治疗后短期预后较好,肺部感染是MPA肺受累的主要死亡原因.

abstractsObjective To explore the clinical features of pulmonary involvement in patients with microscopic polyangiitis(MPA).Methods We retrospectively investigated the clinical data of 50 patients hospitalized with MPA in Peking Union Medical College Hospital from January 2008 to December 2009,the data included clinical manifestation,laboratory parameters,echocardiography,pulmonary funetion test,chest computed tomography,and histopathology of kidney.Results Pulmonary involvements were observed in 46 patients,common symptoms include cough(34/46),expectoration(30/46),dyspnea(19/46)and hemoptysis(16/46).Pulmonary involvement was the initial manifestation in 14 patients,five cases had radiographic evidences of usaal interstitial pneumonia before MPA was diagnosed.The prevalence of positive MPO-ANCA antibodies in MPA patients was 96%.The prevalence of positive PR3-ANCA antibodies was 6% Radiographic manifestations included ground glass attenuation(16/37),interstitial changes(16/37),infihrates(12/37)and pleural effusion(7/37).The most frequent abnormality in pulmonary function test was reduced carbon monoxide diffusing capacity(12/15)and restrictive ventilation dysfunction(4/15).The incidences of pulmonary hypertension was 33%(13/39),the average pulmonary artery systolic pressure was (48±8)mm Hg(1 mm Hg=0.133 kPa).Conclusion The prevalence of pulmonary involvement in patients with MPA was high,pulmonary involvement was the initial manifestation in 28%patients.The clinical manifestations were nonspecific,radiographic manifestations included ground glass attenuation,interstitial changes,infiltrates and pleural effusion.The short term prognosis was well in patients with pulmonary involvement treated with systemic cortieosteroids and cyclophosphamide,infection was a leading cause of death in patients with pulmonary involvement.