Interstitial Lung Disease in Systemic Sclerosis: Lessons Learned from Idiopathic Pulmonary Fibrosis

Abstract

Systemic sclerosis (SSc) has the highest cause–specific mortality of all the connective tissue diseases, and interstitial lung disease (ILD) is the number one cause of death among patients with SSc. Historically, therapeutic agents with widespread immunosuppressive effects have been used to treat SSc-ILD. However, data from recent landmark clinical trials in SSc-ILD have affirmed the prior observation that SSc-ILD is a uniquely heterogenous disease with marked variations in rates of response to immunosuppressive therapy. In the last few years, innovative research studies have advanced our understanding of the pathophysiology of SSc-ILD and stimulated interest in the development of novel candidate therapeutics with potential disease–modifying properties for SSc-ILD. Anti-fibrotics are a new class of drugs that may change our approach to the treatment of SSc-ILD similar to the paradigm shift that occurred for the treatment of idiopathic pulmonary fibrosis (IPF). The present review describes the most recent advances in our understanding of SSc-ILD in the context of lessons learned from the study of IPF. The article concludes with an outline of the most important unanswered questions in SSc-ILD research that could help inform future research efforts in this area.