A 45-year-old woman with no smoking history presents with progressive dyspnea over 3 years. HRCT shows bilateral basilar subpleural reticulation with honeycombing and traction bronchiectasis. Surgical lung biopsy shows usual interstitial pneumonia (UIP) pattern. Which two antifibrotic agents are approved for this condition?
- A N-acetylcysteine and prednisolone
- B Azathioprine and mycophenolate
- C Pirfenidone and nintedanib ✓
- D Cyclophosphamide and rituximab
Correct answer: C. Pirfenidone and nintedanib
Explanation
Idiopathic pulmonary fibrosis (IPF) presents with UIP pattern on HRCT/biopsy and is the most common and most lethal ILD. Two antifibrotic agents—pirfenidone (a pyridone TGF-β signaling inhibitor) and nintedanib (a tyrosine kinase inhibitor blocking VEGFR, PDGFR, FGFR)—are FDA/DCGI-approved for IPF; both reduce the rate of FVC decline by approximately 50% in clinical trials (ASCEND and INPULSIS). The PANTHER trial established that combination of prednisone + azathioprine + NAC was harmful in IPF, increasing mortality. Immunosuppression is not indicated 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.