肺纤维化-肺气肿综合征

定义 英文原文（暂无中文）

A rare interstitial lung disease characterized by the coexistence of emphysema and usual interstitial pneumonia, typically occurring in male smokers. Emphysema is usually encountered in the upper lobes, preceding fibrosis of the lower lobes. Patients present with severe dyspnea and markedly reduced diffusion capacity on functional testing, while spirometric values are relatively preserved. The syndrome is frequently complicated by pulmonary hypertension and acute lung injury.

别名

肺纤维化-肺气肿综合征

基本事实

遗传方式

不适用

发病年龄

成年期

外部标识与链接