Systemic sclerosis related interstitial lung disease and nintedanib

Abstract

Although many mechanisms leading to lung fibrosis in systemic sclerosis-associated interstitial lung disease (SSc-ILD) have been suggested, this issue has not been fully understood yet. Recently, there has been increased evidence that the mediators and pathological mechanisms responsible for idiopathic pulmonary fibrosis (IPF) are similar to those in SSc-ILD. Accordingly, studies have been conducted to support that antifibrotic agents used in the treatment of IPF may also be useful in SSc-ILD. There are currently two antifibrotic agents on the market, namely nintedanib and pirfenidon. Although studies on the use of pirfenidone in SSc-ILD are not satisfactory, nintedanib studies have yielded positive results. The SENSCIS (Safety and Efficacy of Nintedanib in Systemic Sclerosis) study is the first and most comprehensive Phase III study on this subject. In 2019, the results of SENSCIS trial showed that, nintedanib significantly reduced the annual decline in lung function in SSc-ILD. After this trial, which did not include SSc-ILD patients with severe lung function loss, nintedanib licenced for the treatment of SSc-ILD worldwide. However, the currently available literature data lacks information about long-term effects and side effects of nintedanibe on SSc-ILD an also about the advanced SSc-ILD. The aim of this study is to review SSc-ILD patients treated with nintedanib, by also mentioning the pathogenesis of this disease according to the current literature.