A 45-year-old woman presents with 4 months of dyspnoea and dry cough. HRCT chest shows bilateral basal-predominant honeycombing with traction bronchiectasis and a UIP pattern. Surgical lung biopsy shows fibroblastic foci with temporal heterogeneity. The diagnosis is usual interstitial pneumonia (UIP/IPF). According to ATS/ERS/JRS/ALAT 2022 guidelines, which antifibrotic drug reduces the rate of FVC decline?
- A Azathioprine + prednisone + N-acetylcysteine triple therapy
- B Cyclophosphamide
- C Nintedanib ✓
- D Rituximab
Correct answer: C. Nintedanib
Explanation
Nintedanib (and pirfenidone) are the only approved antifibrotic therapies for IPF, both shown in phase III trials (INPULSIS for nintedanib; CAPACITY/ASCEND for pirfenidone) to reduce the rate of FVC decline by approximately 50% annually. The PANTHER-IPF trial showed that triple therapy (prednisone + azathioprine + NAC) was harmful in IPF, increasing mortality and hospitalisation. Cyclophosphamide and rituximab are used in connective tissue disease–associated ILD, not 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.