How is scleroderma pulmonary fibrosis treated?

Updated: Sep 09, 2019
  • Author: Sergio A Jimenez, MD, MACR, FACP, FRCP(UK Hon); Chief Editor: Herbert S Diamond, MD  more...
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Although there is some controversy regarding the beneficial effects of immunosuppressive therapy in idiopathic pulmonary fibrosis, numerous studies support the use of these agents in systemic sclerosis–associated interstitial lung disease. [180, 181] Pulmonary fibrosis in systemic sclerosis has been successfully treated with cyclophosphamide, either orally or in intravenous pulses. [182, 183, 184] Several nonrandomized studies have also shown benefit from mycophenolate mofetil. [143, 144, 145, 185, 186]

Nintedanib was approved by the FDA in September 2019 to slow the rate of decline in pulmonary function in patients who have interstitial lung disease associated with scleroderma. Nintedanib is a tyrosine kinase inhibitor that targets growth factors (eg, vascular endothelial growth factor receptor [VEGFR], fibroblast growth factor receptor [FGFR], platelet-derived growth factor receptor [PDGFR] 1-3, colony-stimulating factor 1 receptor [CSFIR]) that are implicated in the pathogenesis of interstitial lung diseases.

Approval of nintedanib was based on results from the phase 3 SENSCIS trial (n=576). Results showed that nintedanib slowed the loss of pulmonary function by 41 mL/year in patients with systemic sclerosis–related interstitial lung disease relative to placebo, as measured by forced vital capacity (FVC) over a 52- week period. [203]

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