A 55-year-old woman with limited cutaneous systemic sclerosis (lcSSc) on nifedipine for Raynaud's develops progressive dyspnoea. HRCT chest shows bibasilar ground-glass opacities and fine reticulation. PFTs show FVC 68%, DLCO 58%. Bronchoalveolar lavage shows 8% neutrophils and 5% eosinophils. Anti-topoisomerase I (anti-Scl-70) antibody is positive. What is the most appropriate initial disease-modifying therapy?
- A Oral cyclophosphamide
- B High-dose prednisolone 1 mg/kg/day
- C Nintedanib alone
- D Mycophenolate mofetil (MMF) ✓
Explanation
The SLS II trial (Scleroderma Lung Study II) demonstrated that mycophenolate mofetil (MMF) was equivalent to cyclophosphamide for treating SSc-ILD (interstitial lung disease) but with a better tolerability profile. MMF is now the preferred first-line immunosuppressant for SSc-ILD per ACR/EULAR guidelines. Nintedanib (a tyrosine kinase inhibitor) is indicated to slow progression of SSc-ILD (SENSCIS trial) and can be added to or used alongside MMF, but MMF remains the initial immunosuppressive backbone. High-dose steroids are generally avoided in SSc due to risk of precipitating scleroderma renal crisis, particularly above 15 mg prednisolone equivalents.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.