Antifibrotics in systemic sclerosis

Abstract

Systemic sclerosis (SSc) is a rare and complex disease, involving multiple organs, with high morbidity and mortality. Fibrosis is the hallmark of SSc, although vascular and inflammatory mechanisms are also implicated in its pathogenesis. Disease management is challenging, due to its heterogeneous presentation, and to the limited number of controlled clinical trials to guide treating clinicians. Immunosuppressive agents have been used to prevent progression, especially in the lung, before irreversible injury occurs, with some, although modest, benefit. Nintedanib, a tyrosine kinase inhibitor, has recently demonstrated safety and efficacy in interstitial lung disease (ILD) associated with SSc, and many other antifibrotics are being assessed as possible beneficial therapies, with promising results. An important unmet need remains, to clarify to which patients, when, and with which agent therapy should be initiated, to achieve optimal outcomes. This review summarizes available evidence for current and emerging antifibrotic therapies in SSc patients.

Keywords: Antifibrotic therapies; Antifibrotics; Fibrosis; Interstitial lung disease; Scleroderma; Systemic sclerosis.