311 Ch 310. Idiopathic Pulmonary Fibrosis + Specific Interstitial Lung Diseases

IPF = 最常見、最嚴重的 idiopathic ILD；median survival 3-5 years untreated；UIP histopathology + HRCT pattern 為核心；2018 ATS/ERS/JRS/ALAT IPF guideline 強調 HRCT definite/probable UIP 可不必 biopsy；antifibrotic (pirfenidone, nintedanib) 改寫預後 (CAPACITY, ASCEND, INPULSIS)；2020-2024 INBUILD + SENSCIS 將 nintedanib 擴展到 progressive pulmonary fibrosis (PPF) any cause 及 scleroderma-ILD；acute exacerbation IPF = 致命併發症 (30-day mortality > 50%)；lung transplant 是唯一 cure；本章另介紹 NSIP、COP、AIP、PLCH、LAM、PAP、sarcoidosis 詳細，並比較診斷與治療要點。