A 62-year-old woman never-smoker with progressive dyspnoea and dry cough has HRCT showing bilateral basal predominant subpleural reticular opacities with honeycombing and traction bronchiectasis — consistent with UIP pattern. Surgical lung biopsy confirms UIP histology. Pulmonary function tests show FVC 65% predicted, DLCO 48% predicted. Which drug is indicated to slow disease progression?

• A Oral prednisolone 40 mg/day
• B Azathioprine + N-acetylcysteine + prednisolone triple therapy
• C Nintedanib or pirfenidone ✓
• D Cyclophosphamide + mycophenolate

Explanation

This is idiopathic pulmonary fibrosis (IPF) with confirmed UIP pattern. Per ATS/ERS/JRS/ALAT 2022 guidelines, nintedanib (a multi-tyrosine kinase inhibitor) and pirfenidone (an anti-fibrotic with anti-inflammatory and anti-TGF-β activity) are the only approved agents that slow FVC decline (INPULSIS and CAPACITY/ASCEND trials respectively). The PANTHER-IPF trial showed that triple therapy (prednisolone + azathioprine + NAC) increased mortality and hospitalisations and is now contraindicated. Immunosuppressants alone are not beneficial in IPF.

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

High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP

Written and medically reviewed by the StethoPrep medical team.