Lionaki Sophia, Gakiopoulou Hara, Boletis John N
Nephrology Department, Laiko Hospital, Faculty of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
Department of Pathology, Faculty of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
APMIS. 2016 Sep;124(9):725-35. doi: 10.1111/apm.12566. Epub 2016 Jun 30.
An enhanced understanding of the role of complement in the pathogenesis of membranoproliferative glomerulonephritis has led to reclassification of the latter into immunoglobulin-mediated and non-immunoglobulin-mediated disease. The new classification schema resulted in improved diagnostic clinical algorithms, while it brought into light again the diseases, which are characterized by the presence of glomerular deposits, composed predominantly by C3, in the absence of significant amounts of immunoglobulins in renal biopsy, namely, C3 glomerulopathies (dense deposit disease and C3 glomerulonephritis). Despite the lack of randomized controlled trials following the advances in the understanding of the pathogenetic pathways involved in membranoproliferative glomerulonephritis, it is important that the new mechanistic approach has opened new roads for the exploration and discovery of targeted therapies.
对补体在膜增生性肾小球肾炎发病机制中作用的深入理解，已导致后者重新分类为免疫球蛋白介导和非免疫球蛋白介导的疾病。新的分类方案改进了诊断临床算法，同时再次揭示了一些疾病，这些疾病的特征是在肾活检中存在主要由C3组成的肾小球沉积物，而不存在大量免疫球蛋白，即C3肾小球病（致密沉积物病和C3肾小球肾炎）。尽管在对膜增生性肾小球肾炎发病机制的理解取得进展后缺乏随机对照试验，但重要的是，新的机制方法为探索和发现靶向治疗开辟了新途径。
