ABSTRACT

Scleroderma is a polymorphic connective tissue disease comprising diffuse systemic sclerosis, limited scleroderma, linear scleroderma, morphea and CREST syndrome. These clinically well defined but different entities share three common features such as vascular abnormalities, tissue fibrosis and autoimmunity. Though the etiology is not known, it is believed that genetic and environmental factors play a role in the pathogenesis of scleroderma. In humans, it is hard to unravel the underlying cause of scleroderma except in the cases following treatment with bleomycin. Therefore, studies in animal models are important for better understanding of the role of genetic and environmental factors in the development of this disease. Currently available animal models can be grouped into two major categories: 1) inducible and, 2) genetic.