Zhang Yan, Gilliam Anita C
Department of Dermatology, Case Western Reserve University/ University Hospitals of Cleveland, 10900 Euclid Avenue, Cleveland, OH 44106-5028, USA.
Curr Rheumatol Rep. 2002 Apr;4(2):150-62. doi: 10.1007/s11926-002-0011-3.
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.
硬皮病是一种进行性的、使人衰弱的纤维化疾病，可累及多个器官。该疾病的发病机制尚不清楚。硬皮病动物模型对于研究这种复杂疾病的发病机制以及测试人类硬皮病的潜在治疗方法具有重要价值。有几种可用的动物模型表现出硬皮病的重要特征，每种模型都侧重于疾病的不同方面（组织纤维化、炎症、血管损伤或免疫变化）。这些模型可分为几类，其中纤维化可由外部因素（氯乙烯、博来霉素）、通过培育突变株组合（整合素α1基因敲除小鼠、MRL/lprγR-/-小鼠）以及通过移植不同的免疫细胞（硬皮病移植物抗宿主病）诱导产生。此外，还存在自发突变（UCD 200鸡、紧皮小鼠），其中会发生纤维化。紧皮小鼠最近已有综述。本综述讨论了其他动物模型以及每种模型中的一些干预措施。
