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系统性红斑狼疮中的自噬和免疫异常。

Autophagy and immunological aberrations in systemic lupus erythematosus.

机构信息

Renal Division, Peking University First Hospital, Peking University Institute of Nephrology, Key Laboratory of Renal Disease, Ministry of Health of China, Key Laboratory of Chronic Kidney Disease Prevention and Treatment (Peking University), Ministry of Education, Beijing, 100034, People's Republic of China.

出版信息

Eur J Immunol. 2019 Apr;49(4):523-533. doi: 10.1002/eji.201847679. Epub 2019 Feb 25.

DOI:10.1002/eji.201847679
PMID:30776086
Abstract

Systemic lupus erythematosus (SLE) is a complex autoimmune disease, in which immune defects can occur at multiple points of the cascading auto-aggressive immune reactions, resulting in a striking heterogeneity of clinical presentations. The clinical manifestations of such autoimmune response can be severe: common manifestations symptoms include rash and renal inflammation progressing to kidney failure. Autophagy, the cellular "self-digestion" process, is a key factor in the interplay between innate and adaptive immunity. Dysregulation of autophagy has been implicated in numerous autoimmune diseases. Several lines of evidence from genomic studies, cell culture systems, animal models, and human patients are emerging to support the role of autophagy in progression and pathogenesis of SLE. In this review, we summarize recent key findings on the aberrations of autophagy in SLE, with a special focus on how deregulated autophagy promotes autoimmunity and renal damage. We will also discuss how the observed findings may be translated into therapeutic settings.

摘要

系统性红斑狼疮(SLE)是一种复杂的自身免疫性疾病,在级联自身免疫反应的多个点可能发生免疫缺陷,导致临床表现明显异质性。这种自身免疫反应的临床表现可能很严重:常见的表现症状包括皮疹和肾脏炎症进展为肾衰竭。自噬是固有免疫和适应性免疫相互作用的关键因素,细胞的“自我消化”过程。自噬失调与许多自身免疫性疾病有关。来自基因组研究、细胞培养系统、动物模型和人类患者的几条证据线正在出现,以支持自噬在 SLE 的进展和发病机制中的作用。在这篇综述中,我们总结了最近关于 SLE 中自噬异常的关键发现,特别关注失调的自噬如何促进自身免疫和肾脏损伤。我们还将讨论观察到的发现如何转化为治疗环境。

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