A 48-year-old nonsmoking woman presents with progressive dyspnoea over 18 months. HRCT shows peripheral, basal-predominant, bilateral reticular opacities with honeycombing and traction bronchiectasis. BAL shows 3% neutrophils, 2% eosinophils, and 6% lymphocytes. Surgical lung biopsy shows fibroblastic foci with temporal heterogeneity. The MDD (multidisciplinary discussion) diagnoses UIP pattern. Which drug combination has shown benefit in slowing FVC decline in IPF?
- A Combination of nintedanib + pirfenidone as superior dual antifibrotic therapy
- B High-dose N-acetylcysteine triple therapy (NAC + prednisone + azathioprine)
- C Nintedanib or pirfenidone monotherapy (not combination) ✓
- D Mycophenolate mofetil for its antifibrotic properties in IPF
Explanation
Both nintedanib (tyrosine kinase inhibitor targeting PDGFR, VEGFR, FGFR) and pirfenidone (anti-fibrotic, anti-inflammatory) have individually demonstrated reduction in annual FVC decline by approximately 50% in large RCTs (INPULSIS and CAPACITY/ASCEND trials respectively). They are approved as monotherapies for IPF; while INSTAGE and other trials have explored combination, no combination regimen is standard of care — both drugs are used as alternatives, not together. The PANTHER trial showed that triple therapy with NAC + prednisone + azathioprine was actually harmful (increased mortality and hospitalisation) in IPF. MMF is used in CTD-ILD but not IPF.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.