Rationale for the evaluation of nintedanib as a treatment for systemic sclerosis-associated interstitial lung disease.

ABSTRACT: Interstitial lung disease is a common manifestation of systemic sclerosis. Systemic sclerosis-associated interstitial lung disease is characterized by progressive pulmonary fibrosis and a reduction in pulmonary function. Effective treatments for systemic sclerosis-associated interstitial lung disease are lacking. In addition to clinical similarities, systemic sclerosis-associated interstitial lung disease shows similarities to idiopathic pulmonary fibrosis in the pathophysiology of the underlying fibrotic processes. Idiopathic pulmonary fibrosis and systemic sclerosis-associated interstitial lung disease culminate in a self-sustaining pathway of pulmonary fibrosis in which fibroblasts are activated, myofibroblasts accumulate, and the excessive extracellular matrix is deposited. Nintedanib

SUBMITTER: Wollin L 

PROVIDER: S-EPMC8922567 | biostudies-literature | 2019 Oct

REPOSITORIES: biostudies-literature