特发性胸膜实质纤维弹性组织增生

定义 英文原文（暂无中文）

A rare idiopathic interstitial pneumonia characterized by prominent subpleural and parenchymal fibroelastosis and pleural fibrosis, predominantly involving the upper lobes. Signs and symptoms include non-productive cough, dyspnea, and recurrent respiratory infections. Pneumothorax is a frequently reported complication. Pulmonary function test reveals a restrictive pattern and reduced diffusing capacity. Computed tomography shows pleural thickening with signs of fibrosis (traction bronchiectasis, architectural distortion, and loss of volume), and reticulation.

别名

Idiopathic pleuropulmonary fibroelastosis

基本事实

遗传方式

不适用

发病年龄

青少年期、成年期、老年期

患病率

<1 / 1 000 000

外部标识与链接