A 62-year-old non-smoker woman presents with progressive dyspnea. HRCT shows bilateral basilar predominant subpleural honeycombing with traction bronchiectasis. Surgical lung biopsy shows a UIP (usual interstitial pneumonia) pattern with temporal and spatial heterogeneity — areas of normal lung, fibroblast foci, and established fibrosis. The current ATS/ERS/JRS/ALAT 2022 guidelines recommend which first-line anti-fibrotic agent?
- A Prednisolone plus azathioprine as first-line anti-inflammatory therapy for UIP-pattern ILD
- B Nintedanib (triple tyrosine kinase inhibitor targeting PDGFR, VEGFR, FGFR) or pirfenidone (TGF-beta inhibitor/anti-fibrotic); both slow FVC decline but do not reverse fibrosis in IPF ✓
- C N-acetylcysteine monotherapy as an antioxidant to prevent fibrosis progression
- D Mycophenolate mofetil and rituximab for suspected autoimmune ILD even without serologic evidence
Explanation
Idiopathic pulmonary fibrosis (IPF) has a UIP pattern on HRCT and biopsy. The ATS/ERS/JRS/ALAT 2022 guidelines conditionally recommend nintedanib or pirfenidone as the only approved disease-modifying anti-fibrotic therapies that slow but do not reverse fibrosis. Nintedanib inhibits receptor tyrosine kinases involved in fibroblast activation (PDGFR, VEGFR, FGFR), while pirfenidone has broad anti-fibrotic, anti-inflammatory, and antioxidant mechanisms targeting TGF-beta signaling. The 2012 IPF guidelines established that triple therapy with prednisone, azathioprine, and NAC was harmful in IPF and is no longer recommended. Mycophenolate and rituximab are used in autoimmune-associated ILD (e.g., CTD-ILD), not in IPF without autoimmune features.
Reference: Robbins & Cotran Pathologic Basis of Disease, 10th ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.