311.2 🩺 國考版

311.2.1 高頻考點

311.2.1.1 IPF

  • UIP pattern: subpleural + basal + honeycombing + reticular
  • Median survival 3-5 yr untreated
  • Antifibrotic: pirfenidone (CAPACITY, ASCEND), nintedanib (INPULSIS)
  • INBUILD: nintedanib for PPF any cause
  • Acute exacerbation: 30-day mortality > 50%; high-dose steroids debated

311.2.1.2 CTD-ILD vs IPF

  • CTD: younger, female, NSIP > UIP, immunosuppressive
  • IPF: older, male, UIP, antifibrotic

311.2.1.3 NSIP

  • Cellular vs fibrotic
  • CTD-associated 40-60%
  • Better prognosis
  • Immunosuppression

311.2.1.4 COP

  • Migratory consolidation
  • Dramatic steroid response
  • Relapse 30-50%

311.2.1.5 PLCH

  • Almost exclusively smokers
  • Bizarre cysts + upper lobe + nodules
  • Smoking cessation
  • BRAF inhibitor for severe BRAF-mutated

311.2.1.6 LAM

  • Women + childbearing age + TSC
  • Cysts + chylothorax + AML
  • Sirolimus (MILES)
  • Avoid estrogen + pregnancy

311.2.1.7 PAP

  • Anti-GM-CSF (90%)
  • “Crazy paving” HRCT
  • Whole lung lavage + inhaled GM-CSF

311.2.1.8 Sarcoidosis

  • Non-caseating granulomas
  • BAL CD4/CD8 > 4
  • ACE level low sensitivity
  • Löfgren good prognosis
  • Heerfordt syndrome
  • Stages 0-IV (Scadding)
  • Treatment: observe → steroids → steroid-sparing → biologics

311.2.2 易混淆比范

Disease HRCT Key Treatment
IPF UIP (subpleural basal honeycomb) Pirfenidone/nintedanib
NSIP Bilateral GG + reticular IS (MMF + steroids)
COP Migratory consolidation Steroids dramatic
AIP Diffuse GG + consolidation Supportive + steroids
PLCH Upper lobe nodules + cysts Smoking cessation
LAM Diffuse thin-walled cysts Sirolimus
PAP “Crazy paving” Whole lung lavage
Sarcoid Hilar LAD ± parenchymal Steroids ± IS

311.2.3 Special Topics

311.2.3.1 GAP Score for IPF

  • Gender, Age, Physiology (FVC, DLCO)
  • Stage I, II, III
  • Prognosis prediction
  • Useful for transplant decisions

311.2.3.2 Acute Exacerbation IPF Criteria

  • Worsening in < 30 days
  • New ground-glass / consolidation
  • Exclusion of infection, HF, PE
  • Mortality 50%+ in 30 days

311.2.3.3 Hospitalization for IPF

  • Bad prognostic sign
  • ~ 50% die within 6 months
  • Goals of care discussion