A 38-year-old non-smoker woman presents with progressive dyspnoea over 2 years. HRCT thorax shows bilateral basal-predominant reticular opacities with honeycombing and traction bronchiectasis, subpleural distribution without ground-glass opacity. Surgical lung biopsy shows temporal heterogeneity, fibroblastic foci, and honeycombing. What is the diagnosis and the drug approved for this condition?
- A Nonspecific interstitial pneumonia (NSIP); mycophenolate mofetil
- B Cryptogenic organising pneumonia (COP); corticosteroids
- C Usual interstitial pneumonia (UIP)/IPF; nintedanib or pirfenidone ✓
- D Hypersensitivity pneumonitis; antigen avoidance and steroids
Explanation
The clinical, radiological (UIP pattern on HRCT: basal subpleural reticulation, honeycombing, traction bronchiectasis), and histological features (temporal heterogeneity, fibroblastic foci) are diagnostic of idiopathic pulmonary fibrosis (IPF). ATS/ERS guidelines approve two antifibrotic agents — pirfenidone (inhibits TGF-β) and nintedanib (inhibits PDGFR, VEGFR, FGFR) — which slow FVC decline by approximately 50% but do not reverse fibrosis. NSIP shows temporal homogeneity on biopsy and lacks honeycombing. COP has a peribronchovascular/peribronchiolar distribution and responds to steroids.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.