311.4 📋 章末速記 Summary

311.4.1 🔑 一句話總結

IPF = 最常見、最嚴重 idiopathic ILD；UIP pattern + 老年男性 + median survival 3-5 yr untreated；2018 ATS/ERS guidelines 允許 definite UIP on HRCT without biopsy；antifibrotic (pirfenidone CAPACITY/ASCEND, nintedanib INPULSIS) 為標準；INBUILD 2019 將 nintedanib 適應症擴大到 progressive pulmonary fibrosis (PPF) any cause；SENSCIS 2019 for scleroderma-ILD；acute exacerbation = 30-day mortality > 50%；lung transplant 唯一 cure；本章另詳述 NSIP (CTD-associated, IS)、COP (migratory consolidation + dramatic steroid response)、AIP (ARDS-like)、PLCH (smokers + bizarre cysts + BRAF)、LAM (women + TSC + sirolimus MILES)、PAP (anti-GM-CSF + crazy paving + whole lung lavage)、sarcoidosis (non-caseating granulomas + BAL CD4/CD8 > 4 + Scadding stages + Löfgren/Heerfordt)。

311.4.2 💊 治療精要

  • IPF：pirfenidone (anti-TGF-β) 801 TID 或 nintedanib (multi-kinase TKI) 150 BID；slow FVC decline ~ 50%
  • PPF (progressive any cause)：nintedanib (INBUILD 2019) — sclerosis-ILD、HP、CTD-ILD、IPF all eligible
  • scleroderma-ILD：nintedanib + MMF combination；IS for cellular
  • NSIP：steroids + MMF / AZA / rituximab；treat underlying CTD
  • COP：prednisone 0.5-1 mg/kg × 4-12 wk → slow taper 6-12 mo（relapse 30-50%）
  • PLCH：smoking cessation + BRAF inhibitor for severe BRAF-mutated；cladribine refractory
  • LAM：sirolimus MILES；avoid estrogen + pregnancy；pleurodesis for recurrent pneumothorax
  • PAP：whole lung lavage mainstay；inhaled GM-CSF；rituximab refractory
  • sarcoidosis：observation many remit；steroids → MTX/AZA → TNF-α inhibitors / JAK inhibitors

311.4.3 🎯 盧醫師的考前提醒

  1. IPF UIP pattern HRCT 不必 biopsy：sub-pleural + basal + honeycombing + reticular + absence of features inconsistent — 2018 ATS/ERS/JRS/ALAT guidelines
  2. antifibrotic 兩大藥：pirfenidone (anti-TGF-β) vs nintedanib (multi-kinase TKI)；都 slow FVC decline ~ 50% but 不能 reverse fibrosis
  3. INBUILD 2019 改寫範式：nintedanib for progressive pulmonary fibrosis (PPF) any cause — sclerosis-ILD、HP、CTD-ILD、IPF 都可用；PPF definition = ↓ FVC > 5% 或 ↓ DLCO > 10% 或 fibrosis progression
  4. acute exacerbation IPF：< 30 days new dyspnea + new ground-glass + exclude infection/HF/PE；30-day mortality > 50%, 6-month > 70%；high-dose steroids 效果有爭議
  5. GAP score (Gender, Age, Physiology) 預測 IPF prognosis；Stage I-III
  6. NSIP vs IPF 鑑別：NSIP 較常 CTD、bilateral ground-glass、basal/peripheral、no/minimal honeycombing；better prognosis；IS-responsive
  7. COP “migratory consolidation” + dramatic steroid response + relapse 30-50% (slow taper 6-12 mo important)
  8. LAM women + TSC association：cystic lung + chylothorax + renal AML；sirolimus MILES；avoid estrogen + pregnancy（worsens disease）
  9. PAP autoimmune 90%：anti-GM-CSF antibodies；“crazy paving” HRCT pattern（ground-glass + septal thickening）；whole lung lavage mainstay；inhaled GM-CSF + rituximab emerging
  10. sarcoidosis BAL CD4/CD8 > 4（vs HP < 1）；ACE 敏感性低；Löfgren syndrome (erythema nodosum + hilar LAD + arthralgia + fever) = good prognosis often self-limited；Stage I 70-80% 自然 remit