A 52-year-old woman presents with progressive dyspnea over 2 years. HRCT chest shows bilateral basal predominant honeycombing with traction bronchiectasis, subpleural distribution, and no ground glass opacity. Spirometry shows FVC 58% with FEV1/FVC 0.82. DLCO is 40% predicted. BAL shows no lymphocytosis. Surgical lung biopsy shows temporal heterogeneity. Which disease-modifying treatment reduces the rate of FVC decline?
- A Prednisolone 1 mg/kg/day
- B Mycophenolate mofetil
- C Nintedanib or pirfenidone ✓
- D Cyclophosphamide pulse therapy
Explanation
This is idiopathic pulmonary fibrosis (IPF) — UIP pattern on HRCT (honeycombing, subpleural basal predominance, traction bronchiectasis) with temporal heterogeneity on surgical biopsy and restrictive pattern with reduced DLCO. Nintedanib (tyrosine kinase inhibitor targeting PDGFR, VEGFR, FGFR) and pirfenidone (anti-fibrotic with anti-inflammatory effects) are the only two FDA-approved antifibrotic therapies proven to slow FVC decline by approximately 50% compared to placebo in the INPULSIS and CAPACITY/ASCEND trials respectively. Corticosteroids and immunosuppressants are not beneficial in IPF and may cause harm.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.