310 Ch 309. Interstitial Lung Disease — Overview and Classification

ILD (interstitial lung disease) = 200+ 種異質性疾病，影響肺實質 (alveolar walls + interstitium + small airways + vasculature)；分類 (2013 ATS/ERS update)：(1) IIPs (idiopathic interstitial pneumonias) — IPF、NSIP、COP、AIP、RB-ILD、DIP、LIP；(2) CTD-associated ILD — RA、scleroderma、SLE、Sjögren、PM/DM、MCTD；(3) exposure-related — HP、pneumoconioses、drug-induced；(4) granulomatous — sarcoidosis、berylliosis；(5) other — LAM、PLCH、PAP、eosinophilic pneumonia、vasculitis；diagnosis 用 multidisciplinary discussion (MDD) = 臨床 + HRCT pattern + serology + 有需要 cryobiopsy/surgical biopsy；2020-2024 antifibrotic (nintedanib, pirfenidone) 適應症擴大到 progressive pulmonary fibrosis (PPF) any cause (INBUILD)；PF-ILD treatment paradigm 從只治 IPF 變成「progressive 都治」。