310.2 🩺 國考版

310.2.1 高頻考點

310.2.1.1 IIP Categories

  1. IPF (most common, UIP pattern)
  2. NSIP
  3. COP (formerly BOOP)
  4. AIP (Hamman-Rich)
  5. RB-ILD
  6. DIP
  7. LIP

310.2.1.2 IPF

  • UIP pattern: subpleural + basal + honeycombing + reticular
  • Median survival 3-5 yr untreated
  • Antifibrotic: nintedanib + pirfenidone slow FVC decline
  • No improvement; just slowing
  • Lung transplant for select

310.2.1.3 CTD-ILD Associations

  • RA: UIP > NSIP; high mortality
  • Scleroderma: NSIP > UIP; ~ 50% prevalence
  • PM/DM (esp anti-MDA5): NSIP, OP; rapidly progressive
  • Antisynthetase (Jo-1, PL-7, PL-12): NSIP/OP
  • SLE: NSIP, OP, AIP
  • Sjögren: LIP, NSIP

310.2.1.4 Sarcoidosis

  • Non-caseating granulomas
  • BAL CD4/CD8 > 4 (vs HP < 1)
  • ACE level (low sensitivity)
  • Hypercalcemia from vitamin D activation
  • Scadding stages 0-IV
  • Löfgren: erythema nodosum + hilar LAD + arthralgia + fever (good prognosis)
  • Heerfordt: parotid + uveitis + facial nerve + fever

310.2.1.5 COP (formerly BOOP)

  • Migratory consolidation
  • Subacute onset
  • Dramatic steroid response
  • Most recover

310.2.1.6 Drug-Induced ILD

  • Amiodarone, methotrexate, nitrofurantoin, bleomycin
  • ICI pneumonitis (5-10%)
  • Stop drug + steroids if severe

310.2.1.7 Key Trials

  • CAPACITY (2011), ASCEND (2014): pirfenidone for IPF
  • INPULSIS (2014): nintedanib for IPF
  • INBUILD (2019): nintedanib for PPF (progressive pulmonary fibrosis) any cause
  • SENSCIS (2019): nintedanib for scleroderma-ILD
  • MILES (2011): sirolimus for LAM

310.2.1.8 Antifibrotic Side Effects

  • Nintedanib: diarrhea, LFT elevation
  • Pirfenidone: GI, photosensitivity, LFT

310.2.1.9 LAM

  • TSC association
  • Cystic + chylothorax + AML
  • Sirolimus (MILES)
  • Avoid estrogen
  • Women of childbearing age

310.2.1.10 PAP

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

310.2.2 易混淆比范

Disease HRCT Pattern BAL Treatment
IPF UIP (sub-pleural, basal, honeycomb) Macrophages Nintedanib / pirfenidone
NSIP Bilateral ground-glass + reticular Lymphocytic IS (MMF, steroids)
COP Migratory consolidation Lymph + neut Steroids dramatic
Sarcoidosis Hilar LAD ± parenchymal CD4/CD8 > 4 Steroids ± IS
HP (Ch305) Mosaic + centrilobular CD4/CD8 < 1 Antigen avoid + steroids + antifibrotic
LAM Diffuse cysts Normal Sirolimus
PLCH Upper lobe nodules + cysts Langerhans cells Smoking cessation
PAP “Crazy paving” Milky Whole lung lavage

310.2.3 Special Topics

310.2.3.1 Progressive Pulmonary Fibrosis (PPF) — 2022 Definition

  • Non-IPF ILD with progression despite standard treatment
  • Criteria: any of (worsening symptoms, ↓ FVC > 5%, ↓ DLCO > 10%, increased fibrosis on HRCT)
  • Treatment: nintedanib (INBUILD)
  • Shift from “treat IPF only” to “treat progressive any cause”

310.2.3.2 Acute Exacerbation of IPF

  • Sudden worsening (< 30 days)
  • ↑ Mortality (50%+ in 30 days)
  • Exclude infection, HF
  • Treatment: steroids + supportive
  • Antifibrotic doesn’t prevent

310.2.3.3 Lung Transplant in IPF

  • Often considered for FVC < 50% or DLCO < 35%
  • 5-year survival ~ 50%
  • Earlier referral important