Huston D P, Steinberg A D
Yale J Biol Med. 1979 May-Jun;52(3):289-305.
Systemic lupus erythematosus (SLE) is a human autoimmune disease of unknown etiology. Clinical, serologic, immunologic, and pathologic findings are highly variable in different patients and at different times in the same patient. Murine and canine animal models of SLE have been found with clinicopathologic abnormalities resembling those observed in humans. Each animal model has unique characteristics; taken together they reflect the spectrum of disease in human SLE.Investigations in the animals have suggested that genetic, hormonal, immunologic, viral, and other environmental factors contribute to and modify the expression of disease. Where analogous studies are available for humans, the same factors have been found to modify disease expression in a similar fashion. Together, these studies have helped to clarify the multifactorial basis for SLE.The best characterized abnormalities are immunologic. These include excessive B cell function with the formation of large amounts of autoantibodies, and T cell abnormalities which include defects in T cell regulatory function as well as certain T cell effector functions.The animal models of SLE also serve as convenient test subjects for newer therapeutic modalities. It is hoped that further study of the animal models will provide a more rational approach to therapeutic modulation of disease in humans with SLE.
系统性红斑狼疮(SLE)是一种病因不明的人类自身免疫性疾病。临床、血清学、免疫学和病理学表现在不同患者以及同一患者的不同时期差异很大。已发现SLE的小鼠和犬类动物模型存在类似于人类所观察到的临床病理异常。每种动物模型都有其独特的特征;综合起来,它们反映了人类SLE疾病的范围。对动物的研究表明,遗传、激素、免疫、病毒和其他环境因素会促成并改变疾病的表现。在有针对人类的类似研究的情况下,发现相同的因素以类似方式改变疾病表现。这些研究共同有助于阐明SLE的多因素基础。最具特征的异常是免疫方面的。这些包括B细胞功能亢进,形成大量自身抗体,以及T细胞异常,其中包括T细胞调节功能缺陷以及某些T细胞效应功能缺陷。SLE的动物模型也作为新型治疗方法的便利试验对象。希望对动物模型的进一步研究将为治疗人类SLE疾病提供更合理的方法。