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Systemic Sclerosis 2011

L. Chung, O. Distler, L. Hummers, E. Krishnan, V. Steen

International Journal of Rheumatology, 2012

Abstract

Systemic sclerosis (SSc) is a systemic autoimmune disease characterized by widespread fibrosis affecting the skin, internal organs, and vasculature. However, there are currently no systemic, disease-modifying therapies available for the treatment of the overall condition, and the outcomes remain poor. Studies into disease pathogenesis have identified several pathways that are dysregulated in SSc, and novel targeted therapies are currently being developed. In this special issue, we invited authors to submit original research articles, review articles, or case reports/case series describing preclinical, translational, or clinical studies related to new therapies for SSc.

A set of papers in this special issue focuses on identification of new therapeutic targets in preclinical and translational studies. V. J. Moulin's paper is a review article describing the role of apoptosis in the initiation and maintenance of disease in SSc, through effects on the immune system, vascular damage, and fibroblast proliferation. This report discusses potential therapies targeting apoptosis and the Fas/FasL pathway that could be investigated in patients with SSc. Other paper describes vascular changes in the bleomycin-induced mouse model of SSc. The authors discuss the use of this mouse model to investigate targeting fibrosis, apoptosis, and cellular adhesion molecules for the treatment of vascular disease in SSc. The paper by T. Radstake et al. reviews the evidence that hypoxia contributes to the…

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