Emerging targeted therapies in scleroderma lung and skin fibrosis

Abstract

Systemic sclerosis (SSc) is a multisystemic fibrotic disorder that affects the skin and internal organs. Despite an improved outcome probably reflecting a better management of disease complications, morbidity and mortality remain higher than those of patients with other connective tissue diseases. SSc is still considered incurable; however, during recent years, intensive research activities have deepened the understanding of pathogenic mechanisms and have led to the identification of cellular and molecular anti-fibrotic targets. This review article will discuss potential future targeted therapeutic options based on data from in vitro studies, experimental models of fibrosis and first human trials with focus on scleroderma skin and lung fibrosis.