特發性肺纖維化是一種慢性、漸進性纖維化的間質性肺炎，其成因不明，主要發生於年 長者，疾病範圍僅限於肺臟，且此疾病在組織病理學及影像型態上會出現一個特別的變化： 尋常性間質性肺炎(usual interstitial pneumonia, UIP)。要診斷特發性纖維化，病人必須在影像 學或組織病理學上符合尋常性間質性肺炎，且已排除其他可能造成間質性肺炎的原因，像是 環境因子暴露、結締組織疾病或藥物等等。過去一旦診斷特發性肺纖維化，預後非常不好， 在台灣自己的報告中，存活期中位數大約只有0.9 年。隨著近年來對疾病機轉的了解，治療 策略及藥物發展逐漸有了進展，抗纖維化藥物nintedanib 和pirfenidone 的大型研究都顯示可 減緩病人肺功能的惡化程度，目前仍有更多臨床研究在進行中，期待能改善病人的預後。

Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive, fibrotic, and idiopathic interstitial pneumonia. It’s prone to elderlies, and the disease involvement is confined to the lungs. There has a unique pathological findings called usual interstitial pneumonia (UIP) that can be found in both histology and images on computer tomography (CT). To make the diagnosis of IPF, all known-cause of interstitial lung diseases such as environmental factors, medications, connective tissue diseases should be excluded first. In the past, IPF is considered as a poorly prognostic disease, while the median survival time was 0.9 year in Taiwan. However, as the pathogenesis is being studied, anti-fibrotic drugs (nintedanib and pirfenidone) have been developed. They were proved to be effective to decrease lung function decline in some large-scale studies. Moreover, there are more ongoing studies among IPF trying to improve prognosis of IPF patients. (J Intern Med Taiwan 2018; 29: 283-291)