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膜增生性肾小球肾炎的损伤模式。

Membranoproliferative Glomerulonephritis Pattern of Injury.

机构信息

Division of Nephrology, Mount Sinai Hospital, New York, NY.

Division of Nephrology, Mount Sinai Hospital, New York, NY.

出版信息

Adv Kidney Dis Health. 2024 May;31(3):216-222. doi: 10.1053/j.akdh.2024.03.005.

DOI:10.1053/j.akdh.2024.03.005
PMID:39004461
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11251708/
Abstract

Membranoproliferative glomerulonephritis (MPGN) is no longer a disease but a pattern of injury in various diseases. Characterized by electron-dense deposits, mesangial proliferation, and duplication of the glomerular basement membrane, MPGN was previously classified by findings seen by electron microscopy. However, recognizing complement dysfunction in relation to cases with the MPGN pattern of injury substantially changed our view of its pathogenesis. A new classification, including immune complex-mediated and complement-mediated MPGN, has become preferable and has been adopted by international guidelines. Despite these advancements, accurate diagnosis of MPGN remains a clinical challenge, given the pathological and clinical similarities between immune complex-mediated and complement-mediated MPGN. Additional testing, such as molecular and genetic testing, is often necessary. Here, we will summarize our current understanding of the MPGN pattern of injury from a pathology perspective as an introductory article in the following chapters.

摘要

膜增生性肾小球肾炎(MPGN)不再是一种疾病,而是多种疾病的损伤模式。MPGN 的特征是电子致密物沉积、系膜细胞增殖和肾小球基底膜的复制,以前通过电子显微镜检查所见进行分类。然而,认识到补体功能障碍与 MPGN 损伤模式的关系,极大地改变了我们对其发病机制的看法。一种新的分类,包括免疫复合物介导和补体介导的 MPGN,已经变得更加可取,并被国际指南所采用。尽管取得了这些进展,但由于免疫复合物介导和补体介导的 MPGN 在病理和临床方面具有相似性,因此准确诊断 MPGN 仍然是一个临床挑战。通常需要进行额外的测试,如分子和基因测试。在这里,我们将从病理学角度总结我们目前对 MPGN 损伤模式的理解,作为以下章节的介绍性文章。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e04b/11251708/9a5f68286d73/nihms-1980094-f0004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e04b/11251708/7ed91b44e48e/nihms-1980094-f0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e04b/11251708/fc429ca28a10/nihms-1980094-f0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e04b/11251708/ae17c702d700/nihms-1980094-f0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e04b/11251708/9a5f68286d73/nihms-1980094-f0004.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e04b/11251708/7ed91b44e48e/nihms-1980094-f0001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e04b/11251708/fc429ca28a10/nihms-1980094-f0002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e04b/11251708/ae17c702d700/nihms-1980094-f0003.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/e04b/11251708/9a5f68286d73/nihms-1980094-f0004.jpg

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本文引用的文献

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Iptacopan in Idiopathic Immune Complex-Mediated Membranoproliferative Glomerulonephritis: Protocol of the APPARENT Multicenter, Randomized Phase 3 Study.依帕卡潘治疗特发性免疫复合物介导的膜增生性肾小球肾炎:APPARENT多中心随机3期研究方案
Kidney Int Rep. 2023 Oct 29;9(1):64-72. doi: 10.1016/j.ekir.2023.10.022. eCollection 2024 Jan.
2
The Uncertainty Puzzle of Monoclonal Gammopathy of Renal Significance Without Detectable Clones.隐匿克隆的具有肾脏意义的单克隆丙种球蛋白病的不确定性难题
Kidney Int Rep. 2023 Oct 18;8(12):2511-2514. doi: 10.1016/j.ekir.2023.10.013. eCollection 2023 Dec.
3
Overlap of C3 Glomerulopathy and Thrombotic Microangiopathy: A Case Series.
C3肾小球病与血栓性微血管病的重叠:病例系列
Kidney Int Rep. 2022 Dec 24;8(3):619-627. doi: 10.1016/j.ekir.2022.12.009. eCollection 2023 Mar.
4
Membranoproliferative glomerulonephritis: no longer the same disease and may need very different treatment.膜增生性肾小球肾炎:不再是同一种疾病,可能需要非常不同的治疗。
Nephrol Dial Transplant. 2023 Feb 13;38(2):283-290. doi: 10.1093/ndt/gfab281.
5
KDIGO 2021 Clinical Practice Guideline for the Management of Glomerular Diseases.KDIGO 2021肾小球疾病管理临床实践指南。
Kidney Int. 2021 Oct;100(4S):S1-S276. doi: 10.1016/j.kint.2021.05.021.
6
Immunotactoid glomerulopathy is a rare entity with monoclonal and polyclonal variants.免疫触须样肾小球病是一种罕见的疾病,有单克隆和多克隆变异型。
Kidney Int. 2021 Feb;99(2):410-420. doi: 10.1016/j.kint.2020.07.037. Epub 2020 Aug 18.
7
Practical management of C3 glomerulopathy and Ig-mediated MPGN: facts and uncertainties.C3肾小球病和Ig介导的膜增生性肾小球肾炎的实际管理:事实与不确定性
Kidney Int. 2020 Nov;98(5):1135-1148. doi: 10.1016/j.kint.2020.05.053. Epub 2020 Jul 3.
8
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Kidney Int. 2020 Mar;97(3):589-601. doi: 10.1016/j.kint.2019.10.025. Epub 2019 Nov 9.
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