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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Answer

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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