310.4 📋 章末速記 Summary

310.4.1 🔑 一句話總結

ILD = 200+ heterogeneous disorders affecting lung parenchyma；分類 (2013 ATS/ERS)：(1) IIPs (IPF、NSIP、COP、AIP、RB-ILD、DIP、LIP)；(2) CTD-associated (RA、scleroderma、PM/DM 抗 MDA5 rapidly progressive、antisynthetase、SLE、Sjögren)；(3) exposure (HP Ch305、pneumoconioses Ch306、drug-induced)；(4) granulomatous (sarcoidosis - non-caseating + BAL CD4/CD8 > 4 + Löfgren/Heerfordt syndromes、berylliosis)；(5) other (LAM + TSC + sirolimus、PLCH + smokers + BRAF、PAP + anti-GM-CSF + crazy paving + whole lung lavage)；IPF treatment = antifibrotic (nintedanib, pirfenidone) slow FVC decline；INBUILD 2019 將 nintedanib 適應症擴大到 progressive pulmonary fibrosis (PPF) any cause；MDD (multidisciplinary discussion) 是 ILD diagnosis gold standard。

310.4.2 💊 治療精要

• IPF：nintedanib (tyrosine kinase inhibitor, multi-target PDGF/FGF/VEGF) 或 pirfenidone (anti-TGF-β)；slow FVC decline ~ 50%；lung transplant for end-stage
• NSIP / CTD-ILD：immunosuppression（steroids + MMF / AZA / rituximab）；nintedanib for progressive (INBUILD/SENSCIS)
• COP：oral prednisone 0.5-1 mg/kg → taper；dramatic response
• sarcoidosis：observation (many remit) OR steroids (symptomatic/progressive)；MTX、AZA、TNF-α inhibitors (refractory)；JAK inhibitor emerging
• LAM：sirolimus (MILES trial)；avoid estrogen；lung transplant for end-stage
• PAP：whole lung lavage (mainstay)；inhaled GM-CSF；rituximab
• drug-induced：stop offending agent + steroids if severe
• MDD：clinician + radiologist + pathologist consensus diagnosis

310.4.3 🎯 盧醫師的考前提醒

1. IPF UIP pattern HRCT：subpleural + basal predominance + reticular + honeycombing + traction bronchiectasis；definite UIP 不必 biopsy
2. antifibrotic 兩大藥：pirfenidone (anti-TGF-β) + nintedanib (multi-kinase TKI)；都 slow FVC decline，不能 reverse；side effects pirfenidone = photosensitivity + GI，nintedanib = diarrhea + LFT
3. INBUILD 2019 改變治療範式：nintedanib for progressive pulmonary fibrosis (PPF) any cause — 不只 IPF；PPF definition = ↓ FVC > 5% 或 ↓ DLCO > 10% 或 fibrosis progression on HRCT
4. NSIP vs IPF：NSIP 較常 CTD-associated、predominantly ground-glass、basal/peripheral；better prognosis than IPF；immunosuppression 為主
5. COP (formerly BOOP) 特徵：migratory consolidation + subacute + dramatic steroid response + most recover；relapse rate 30-50%
6. Sarcoidosis 兩大綜合徵：Löfgren (erythema nodosum + bilateral hilar LAD + arthralgia + fever — good prognosis, often self-limited)；Heerfordt (parotid + uveitis + facial nerve + fever)
7. Sarcoidosis BAL vs HP：sarcoid CD4/CD8 > 4；HP CD4/CD8 < 1（鑑別兩者重要）
8. anti-MDA5 + rapidly progressive ILD in PM/DM：aggressive IS（cyclophosphamide + rituximab + tofacitinib + plasmapheresis）；mortality 高
9. LAM + TSC + sirolimus (MILES 2011)：young women + cystic lung + chylothorax + renal AML；avoid estrogen；lung transplant for end-stage
10. PAP “crazy paving” HRCT：anti-GM-CSF antibodies (90%, autoimmune)；whole lung lavage 標準治療；inhaled GM-CSF + rituximab emerging