A patient with diffuse cutaneous systemic sclerosis (dcSSc) has Rodnan skin score 30, FVC 62% predicted, DLCO 41%, and HRCT showing extensive honeycombing and traction bronchiectasis. Which treatment has the strongest evidence for slowing progression of SSc-ILD?
- A Mycophenolate mofetil based on SLS-I and SLS-II trial data
- B D-penicillamine for anti-fibrotic effect on both skin and lung
- C Cyclophosphamide as first-line based on SLS-I superiority over MMF
- D Nintedanib based on SENSCIS trial data ✓
Explanation
SENSCIS (NEJM 2019) showed that nintedanib (a tyrosine kinase inhibitor targeting PDGFR, FGFR, VEGFR) significantly reduced the annual rate of decline in FVC in SSc-ILD (−52.4 vs. −93.3 mL/year), receiving FDA approval for this indication. Mycophenolate (SLS-II, NEJM 2016) was non-inferior to cyclophosphamide for stabilising FVC with a better safety profile, making MMF an immunosuppressive standard of care. Nintedanib is now added to MMF or used in patients with progressive-fibrosing ILD. D-penicillamine has largely fallen out of favour. SLS-I showed cyclophosphamide > placebo; SLS-II showed MMF = cyclophosphamide.
Reference: Harrison's Principles of Internal Medicine, 21st ed.
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Written and medically reviewed by the StethoPrep medical team.