An update on the pharmacotherapeutic options and treatment strategies for systemic sclerosis

Abstract

Introduction: Systemic sclerosis (SSc) is a multi-dimensional connective tissue disease of unknown etiology. Given the immense clinical complexity of SSc, the treatment of this condition is not standardized and considerable heterogeneity exists in SSc management approaches. The purpose of this article is to highlight novel therapeutic strategies and new medications under development for the treatment of systemic sclerosis (SSc).

Areas covered: Herein, the authors focus primarily on recently completed clinical trials and phase 3 and 4 clinical trials of therapeutic agents that show promise in SSc. This review is organized by the clinical complications that occur in SSc, for which novel treatment strategies are under study.

Expert opinion: Combining therapies to address the individual manifestations of SSc is a cornerstone to the comprehensive management of this condition. Therapeutic strategies must take into account the organs involved, the level of disease activity in each area, and the disease stage. Controlling the complex biological network, progressive vasculopathy and fibrosis, as well as manifestations of end-organ dysfunction are all critical considerations when determining the best treatment approach for SSc.

Keywords: Scleroderma; systemic sclerosis; therapy; treatment; update.

Figure 1.

Drugs targeting specific complications of systemic sclerosis in the skin, lungs, peripheral vasculature, pulmonary arteries, and gastrointestinal tract.