中华结核和呼吸杂志
中華結覈和呼吸雜誌
중화결핵화호흡잡지
Chinese Journal of Tuberculosis and Respiratory Diseases
2011年
5期
339-343
,共5页
靳建军%施举红%陆慰萱%朱元珏
靳建軍%施舉紅%陸慰萱%硃元玨
근건군%시거홍%륙위훤%주원각
肺疾病,间质性%高血压,肺性%显微镜下多血管炎
肺疾病,間質性%高血壓,肺性%顯微鏡下多血管炎
폐질병,간질성%고혈압,폐성%현미경하다혈관염
Lung disease,interstitial%Hypertension,pulmonary%Microscopic polyangiitis
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.
目的 探討顯微鏡下多血管炎(microscopic polyangiitis,MPA)肺部病變特點.方法 迴顧性分析2008年1月至2009年12月在北京協和醫院偉院的50例MPA患者的臨床資料,包括臨床錶現、實驗室檢查、超聲心動圖、肺功能、胸部CT及腎髒病理等.結果 男22例,女28例,年齡23～85歲,平均(66±12)歲.病程中存在肺部病變者46例(92%),常見癥狀有咳嗽(34/46,74%)、咳痰(30/46,65%)、呼吸睏難(19/46,41%)和咯血(16/46,35%);14例(14/50,28%)以肺部病變為首髮錶現,其中5例影像學特點符閤普通型問質性肺炎.抗中性粒細胞胞漿抗體(ANCA)中髓過氧化物酶(MPO)-ANCA暘性率96%(48/50),蛋白酶3(PR3)-ANCA暘性率6%(3/50).影像學錶現為磨玻璃影者16例,肺間質纖維化16例,浸潤影12例,胸腔積液7例.常見肺功能異常為瀰散功能障礙(12/15)和限製性通氣功能障礙(4/15).肺動脈高壓13例,肺動脈收縮壓平均為(48±8)mmHg(1 mm Hg=0.133 kPa).結論 MPA肺受纍髮生率高,約1/3患者以肺受纍為首髮錶現;臨床錶現無特異性,磨玻璃影、肺間質纖維化、浸潤影和胸腔積液為常見影像學錶現;激素和免疫抑製劑治療後短期預後較好,肺部感染是MPA肺受纍的主要死亡原因.
목적 탐토현미경하다혈관염(microscopic polyangiitis,MPA)폐부병변특점.방법 회고성분석2008년1월지2009년12월재북경협화의원위원적50례MPA환자적림상자료,포괄림상표현、실험실검사、초성심동도、폐공능、흉부CT급신장병리등.결과 남22례,녀28례,년령23～85세,평균(66±12)세.병정중존재폐부병변자46례(92%),상견증상유해수(34/46,74%)、해담(30/46,65%)、호흡곤난(19/46,41%)화각혈(16/46,35%);14례(14/50,28%)이폐부병변위수발표현,기중5례영상학특점부합보통형문질성폐염.항중성립세포포장항체(ANCA)중수과양화물매(MPO)-ANCA양성솔96%(48/50),단백매3(PR3)-ANCA양성솔6%(3/50).영상학표현위마파리영자16례,폐간질섬유화16례,침윤영12례,흉강적액7례.상견폐공능이상위미산공능장애(12/15)화한제성통기공능장애(4/15).폐동맥고압13례,폐동맥수축압평균위(48±8)mmHg(1 mm Hg=0.133 kPa).결론 MPA폐수루발생솔고,약1/3환자이폐수루위수발표현;림상표현무특이성,마파리영、폐간질섬유화、침윤영화흉강적액위상견영상학표현;격소화면역억제제치료후단기예후교호,폐부감염시MPA폐수루적주요사망원인.
Objective 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.
