特發性肺纖維化

Idiopathic Pulmonary Fibrosis

吳常瑋(Chang-Wei Wu) ； 張皓鈞(Hao-Chun Chang) ； 陳天華(Tien-Hua Chen) ； 黃信端(Hsin-Tuan Huang) ； 劉家榮(Chia-Jung Liu) ； 王鶴健(Hao-Chien Wang)

《內科學誌》 29卷5期 (2018/10) Pp. 283-291

https://doi.org/10.6314/JIMT.201810_29(5).02

摘要

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。

關鍵字

肺纖維化(Lung fibrosis) ； 間質性肺病(Interstitial lung disease)

並列摘要

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.