Division of Nephrology, Mount Sinai Hospital, New York, NY.
Division of Nephrology, Mount Sinai Hospital, New York, NY.
Adv Kidney Dis Health. 2024 May;31(3):216-222. doi: 10.1053/j.akdh.2024.03.005.
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.
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。