A 52-year-old woman with systemic sclerosis (diffuse cutaneous SSc) presents with new onset dyspnoea and progressive exertional limitation over 3 months. HRCT shows basal-predominant fine reticulation with ground-glass opacity. Spirometry: FVC 62% predicted, DLCO 48% predicted. What is the current evidence-based first-line treatment for SSc-ILD?
- A Methotrexate 15 mg weekly
- B Nintedanib (antifibrotic) or mycophenolate mofetil based on SENSCIS and SLS II trial evidence ✓
- C High-dose prednisolone 60 mg daily for 6 months
- D Cyclophosphamide pulse therapy as the only proven treatment
Explanation
The SENSCIS trial demonstrated that nintedanib significantly slowed FVC decline in SSc-ILD. The Scleroderma Lung Study II (SLS II) showed mycophenolate mofetil was as effective as cyclophosphamide with a better safety profile, making MMF the preferred immunosuppressant. Both nintedanib and MMF are now first-line options. High-dose steroids are avoided in SSc due to risk of scleroderma renal crisis.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.