Animal models for scleroderma: an update

Scleroderma is a progressive debilitating fibrosing disease that may involve multiple organs. The pathogenesis of this disease remains unclear. Animal models for scleroderma are valuable for studying the pathogenesis of this complex disorder and for testing potential treatments for human scleroderma. There are several animal models available that exhibit important features of scleroderma, each with an emphasis on different aspects of the disease (tissue fibrosis, inflammation, vascular injury, or immunologic changes). These models can be separated into several categories in which fibrosis is induced by external agents (vinyl chloride, bleomycin), by breeding of mutant strain combinations (integrin alpha 1 null mouse, MRL/lpr gamma R-/- mouse), and by transplantation of disparate immune cells (sclerodermatous graft versus host disease). In addition, there are spontaneous mutations (UCD 200 chicken, tight skin mouse) in which fibrosis occurs. The tight skin mouse has been reviewed recently. This review discusses the other animal models and some interventions in each.