Jin Jian-jun, Shi Ju-hong, Lu Wei-xuan, Zhu Yuan-jue
Department of Respiratory Medicine, Chinese Academy of Medical Science, Beijing 100730, China.
Zhonghua Jie He He Hu Xi Za Zhi. 2011 May;34(5):339-43.
To explore the clinical features of pulmonary involvement in patients with microscopic polyangiitis (MPA).
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 function test, chest computed tomography, and histopathology of kidney.
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 usual 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), infiltrates (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).
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 corticosteroids and cyclophosphamide, infection was a leading cause of death in patients with pulmonary involvement.
探讨显微镜下多血管炎（MPA）患者肺部受累的临床特征。
回顾性分析2008年1月至2009年12月在北京协和医院住院治疗的50例MPA患者的临床资料，包括临床表现、实验室指标、超声心动图、肺功能检查、胸部计算机断层扫描及肾脏组织病理学检查。
46例患者出现肺部受累，常见症状包括咳嗽（34/46）、咳痰（30/46）、呼吸困难（19/46）和咯血（16/46）。14例患者肺部受累为首发表现，5例在MPA诊断前有普通型间质性肺炎的影像学证据。MPA患者中MPO-ANCA抗体阳性率为96%，PR3-ANCA抗体阳性率为6%。影像学表现包括磨玻璃影（16/37）、间质改变（16/37）、浸润影（12/37）和胸腔积液（7/37）。肺功能检查最常见的异常是一氧化碳弥散量降低（12/15）和限制性通气功能障碍（4/15）。肺动脉高压发生率为33%（13/39），平均肺动脉收缩压为（48±8）mmHg（1mmHg = 0.133kPa）。
MPA患者肺部受累发生率高，28%患者肺部受累为首发表现。临床表现无特异性，影像学表现包括磨玻璃影、间质改变、浸润影和胸腔积液。接受全身糖皮质激素和环磷酰胺治疗的肺部受累患者短期预后良好，感染是肺部受累患者的主要死亡原因。
