Animal models of systemic sclerosis

Abstract

Systemic sclerosis (scleroderma) is a fibrotic condition characterized by immunologic abnormalities, vascular injury, and increased accumulation of extracellular matrix proteins in the affected organs. The etiology of scleroderma has not yet been fully elucidated, and thus satisfactory therapeutic drugs are still limited. However, there are two definite and easily available mouse models so far, without genetic modification, tight skin mouse, and bleomycin-induced murine scleroderma. Making use of those mice, many research projects have been performed for a better understanding of the pathophysiology of, and exploring effective therapies for, scleroderma. Furthermore, a number of transgenic or gene-deficient mice are also produced, which mimic human scleroderma. They reproduce several, but not all, histological as well as biochemical aspects resembling human scleroderma, and we can learn lots of new findings through animal studies. This paper introduces current concepts of various animal models for scleroderma and discusses several new aspects of scleroderma pathogenesis and therapeutic approach for scleroderma.