A 45-year-old non-smoker woman presents with progressive dyspnoea, dry cough, and bilateral basal crackles for 18 months. HRCT shows bilateral subpleural, basal predominant honeycombing with traction bronchiectasis. Surgical lung biopsy shows UIP pattern. What is the diagnosis and first-line antifibrotic treatment?

• A Hypersensitivity pneumonitis; oral prednisolone
• B Non-specific interstitial pneumonia; cyclophosphamide
• C Cryptogenic organising pneumonia; high-dose steroids
• D Idiopathic pulmonary fibrosis; nintedanib or pirfenidone ✓

Explanation

The clinical and HRCT features (subpleural honeycombing, basal predominance) with biopsy-confirmed UIP pattern establish IPF. Both nintedanib (INPULSIS trial) and pirfenidone (ASCEND, CAPACITY trials) slow FVC decline by approximately 50% and are ATS/ERS 2022 conditional recommendations. Antifibrotics do not reverse fibrosis but slow progression. Hypersensitivity pneumonitis has an upper-lobe, peribronchovascular distribution and responds to antigen avoidance ± steroids. COP responds dramatically to steroids. NSIP has subpleural sparing and ground-glass predominance.

Reference: Harrison's Principles of Internal Medicine, 21st ed.

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.