Department of Medicine, University of Florida, Gainesville, FL, USA.
Department of Microbiology, Immunology, and Molecular Genetics, UT Health, San Antonio, TX, USA.
Autoimmunity. 2024 Dec;57(1):2319203. doi: 10.1080/08916934.2024.2319203. Epub 2024 Mar 13.
Lupus nephritis (LN) is the most severe end-organ pathology in Systemic Lupus Erythematosus (SLE). Research has enhanced our understanding of immune effectors and inflammatory pathways in LN. However, even with the best available therapy, the rate of complete remission for proliferative LN remains below 50%. A deeper understanding of the resistance or susceptibility of renal cells to injury during the progression of SLE is critical for identifying new targets and developing effective long-term therapies. The complex and heterogeneous nature of LN, combined with the limitations of clinical research, make it challenging to investigate the aetiology of this disease directly in patients. Hence, multiple murine models resembling SLE-driven nephritis are utilised to dissect LN's cellular and genetic mechanisms, identify therapeutic targets, and screen novel compounds. This review discusses commonly used spontaneous and inducible mouse models that have provided insights into pathogenic mechanisms and long-term maintenance therapies in LN.
狼疮肾炎(LN)是系统性红斑狼疮(SLE)中最严重的终末器官病变。研究已经增强了我们对 LN 中免疫效应物和炎症途径的理解。然而,即使采用了最佳的现有治疗方法,增生性 LN 的完全缓解率仍低于 50%。深入了解 SLE 进展过程中肾脏细胞对损伤的抵抗或易感性,对于确定新的靶点和开发有效的长期治疗方法至关重要。LN 的复杂性和异质性,加上临床研究的局限性,使得直接在患者中研究该疾病的病因变得具有挑战性。因此,多种类似于 SLE 驱动的肾炎的小鼠模型被用于剖析 LN 的细胞和遗传机制、确定治疗靶点和筛选新型化合物。本文综述了常用于研究 LN 的自发性和诱导性小鼠模型,这些模型为 LN 的发病机制和长期维持治疗提供了新的见解。