Lupus Nephritis: Animal Modeling of a Complex Disease Syndrome Pathology.
Lupus Nephritis: Animal Modeling of a Complex Disease Syndrome Pathology.
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DOI：
10.1016/j.ddmod.2014.08.002
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发表时间：
2014
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自身免疫引起的肾炎是与系统性红斑狼疮 (SLE) 相关的常见疾病。临床和行业对 SLE 肾病过程的药物干预具有浓厚的兴趣；然而，专门治疗狼疮性肾炎的临床试验尚未使所有患者得到完全和持续的缓解。多种小鼠模型已被用来研究自身免疫反应性与 SLE 病理学之间的病理相互作用。虽然几种模型与 SLE 驱动的肾炎具有显着的相似性，但每种模型都存在局限性，这使得为 SLE 病理学的特定方面选择合适模型的任务具有挑战性。考虑到人类疾病的多变性和多样性，这并不奇怪。在许多方面，小鼠品系的特征模仿了狼疮患者的一些（但不是全部）自身免疫和病理特征。尽管多样性常常限制与发病机制相关的普遍结论，但它们提供了对导致肾炎表型表现的复杂过程的见解。因此，肾炎代表了全身性疾病的一个缩影，具有不同的病变和临床特征。在这篇综述中，我们讨论了一些最常用的狼疮性肾炎 (LN) 模型和免疫介导的肾小球损伤模型，检查它们的相对优点和缺点，这可能有助于了解人类状况。
Nephritis as a result of autoimmunity is a common morbidity associated with Systemic Lupus Erythematosus (SLE). There is substantial clinical and industry interest in medicinal intervention in the SLE nephritic process; however, clinical trials to specifically treat lupus nephritis have not resulted in complete and sustained remission in all patients. Multiple mouse models have been used to investigate the pathologic interactions between autoimmune reactivity and SLE pathology. While several models bear a remarkable similarity to SLE-driven nephritis, there are limitations for each that can make the task of choosing the appropriate model for a particular aspect of SLE pathology challenging. This is not surprising given the variable and diverse nature of human disease. In many respects, features among murine strains mimic some (but never all) of the autoimmune and pathologic features of lupus patients. Although the diversity often limits universal conclusions relevant to pathogenesis, they provide insights into the complex process that result in phenotypic manifestations of nephritis. Thus nephritis represents a microcosm of systemic disease, with variable lesions and clinical features. In this review, we discuss some of the most commonly used models of lupus nephritis (LN) and immune-mediated glomerular damage examining their relative strengths and weaknesses, which may provide insight in the human condition.