A 50-year-old woman presents with progressive dyspnoea, dry cough for 18 months, and bilateral basal crackles. HRCT shows bilateral subpleural, basal-predominant reticular pattern with honeycombing and traction bronchiectasis. BAL lymphocytosis is absent. The diagnosis and its implication for pharmacotherapy is:

• A Non-specific interstitial pneumonia (NSIP); high-dose immunosuppression is first-line
• B Usual interstitial pneumonia (UIP)/IPF pattern; antifibrotics (pirfenidone or nintedanib) are indicated ✓
• C Hypersensitivity pneumonitis (HP); antigen avoidance + corticosteroids are curative
• D Cryptogenic organising pneumonia (COP); excellent response to prednisolone expected

Explanation

The HRCT pattern of bilateral subpleural honeycombing with basal-predominant reticular changes and traction bronchiectasis is the classical UIP pattern pathognomonic of IPF when other causes are excluded. Per ATS/ERS/JRS/ALAT 2022 guidelines, antifibrotic therapy — either pirfenidone (anti-inflammatory/antifibrotic) or nintedanib (multi-kinase inhibitor) — slows FVC decline and is strongly recommended. NSIP is associated with ground-glass predominance without honeycombing; COP shows consolidation in a peribronchovascular distribution.

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

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Written and medically reviewed by the StethoPrep medical team.