A 44-year-old woman with limited cutaneous systemic sclerosis (lcSSc) presents with worsening dyspnoea. HRCT shows ground-glass opacities and fine reticulation in both lower lobes. PFTs reveal FVC 64% predicted. What is the current evidence-based first-line disease-modifying treatment for SSc-ILD?
- A Nintedanib
- B Mycophenolate mofetil ✓
- C Cyclophosphamide IV pulse
- D Pirfenidone
Explanation
The SLS II trial demonstrated that mycophenolate mofetil (MMF) was equivalent to cyclophosphamide for SSc-ILD with better tolerability and a more favourable adverse-effect profile, making it current first-line treatment per ACR and EULAR guidelines. Nintedanib (SENSCIS trial) slows FVC decline and is approved as an add-on or alternative, particularly in progressive SSc-ILD. Pirfenidone lacks evidence in SSc-ILD. IV cyclophosphamide remains an alternative but is now second-line given MMF equivalence.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
High-yield for: NEET PGINI-CETNExTFMGEUSMLEPLABMRCP
Written and medically reviewed by the StethoPrep medical team.