A 45-year-old non-smoker woman presents with progressive dyspnoea over 18 months. HRCT shows bilateral basilar predominant reticular opacities with honeycombing and subpleural distribution. Bronchoalveolar lavage shows no lymphocytosis. Surgical lung biopsy reveals fibroblastic foci with temporal heterogeneity and honeycombing. Diagnosis is UIP/IPF. According to ATS/ERS IPF 2022 guidelines, which treatment is preferred for mild-moderate IPF?

• A Prednisolone 40 mg/day and azathioprine combination (immunosuppressive therapy)
• B N-acetylcysteine monotherapy
• C Cyclophosphamide and mycophenolate mofetil
• D Nintedanib (triple tyrosine kinase inhibitor) or pirfenidone (anti-fibrotic) ✓

Explanation

Two anti-fibrotic agents are approved for IPF: nintedanib (inhibits PDGFR, FGFR, VEGFR) and pirfenidone (TGF-β pathway inhibition, anti-fibrotic, anti-inflammatory). Both the INPULSIS (nintedanib) and CAPACITY/ASCEND (pirfenidone) trials demonstrated significant slowing of FVC decline. They are recommended for mild-to-moderate IPF (FVC >50%, DLCO >30%). The PANTHER-IPF trial definitively showed that prednisone + azathioprine + NAC increases mortality vs placebo, so immunosuppression is contraindicated. NAC monotherapy showed no benefit in the PANTHER trial.

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

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Written and medically reviewed by the StethoPrep medical team.