A 40-year-old non-smoker woman develops progressive dyspnoea over 18 months. HRCT shows bilateral basal-predominant reticular opacities with honeycombing and traction bronchiectasis. Surgical lung biopsy shows UIP pattern. No identifiable cause found. What is the diagnosis and current management recommendation?

• A Nonspecific interstitial pneumonia (NSIP); oral prednisolone 1 mg/kg/day
• B Idiopathic pulmonary fibrosis; nintedanib or pirfenidone to slow progression ✓
• C Hypersensitivity pneumonitis; avoidance of antigen plus azathioprine
• D Cryptogenic organising pneumonia; high-dose corticosteroids for 6 months

Explanation

Idiopathic pulmonary fibrosis (IPF) is diagnosed by UIP pattern on HRCT or histology in the absence of an identifiable cause. ATS/ERS/JRS/ALAT 2022 guidelines strongly recommend antifibrotic therapy — nintedanib (tyrosine kinase inhibitor) or pirfenidone — which slow FVC decline by approximately 50% versus placebo. Corticosteroids are not recommended and may harm IPF patients. NSIP shows ground-glass opacity pattern without honeycombing; COP has nodular/consolidative pattern. UIP pattern with honeycombing is pathognomonic for IPF.

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

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Written and medically reviewed by the StethoPrep medical team.