Hohenstein B, Amann K, Menne J
Nephrologisches Zentrum Villingen-Schwenningen, Albert-Schweitzer‑Str. 6, 78052, Villingen-Schwenningen, Deutschland.
Medizinische Fakultät Carl Gustav Carus, TU Dresden, Dresden, Deutschland.
Internist (Berl). 2019 May;60(5):458-467. doi: 10.1007/s00108-019-0572-0.
Based on an increasingly better pathophysiological understanding over the last 10 years, in 2010 a new classification of glomerulonephritis with dominant or codominant C3 deposits was introduced and the predominant subgoup was termed C3 glomerulopathy (C3G). In the current classification, immune complex mediated membranoproliferative glomerulonephritis (IC-MPGN) and C3 glomerulopathy (C3G) form a disease spectrum which is very heterogeneous in terms of pathophysiology and the clinical course. Recent evidence suggests that IC-MPGN and C3G share more pathophysiological aspects with respect to secondary causes, autoantibodies and genetic aspects than had been suggested with the creation of the new classification. Knowledge of the underlying pathophysiology is important for guiding the diagnostic steps for clarification of secondary causes. Comprehensive complement analysis, accompanied by antibody screening and genetic analysis, should be consistently carried out. Although not systematically validated in clinical trials, the published evidence provides a robust foundation for the use of available treatment approaches for these diseases that are often rapidly progressive and often return after renal transplantation.
基于过去10年对病理生理学的认识日益加深，2010年引入了一种新的以C3沉积为主或共显性的肾小球肾炎分类，主要亚组被称为C3肾小球病（C3G）。在当前分类中，免疫复合物介导的膜增生性肾小球肾炎（IC-MPGN）和C3肾小球病（C3G）构成了一个疾病谱，在病理生理学和临床病程方面非常异质性。最近的证据表明，IC-MPGN和C3G在继发原因、自身抗体和遗传方面共享的病理生理方面比新分类创建时所表明的更多。了解潜在的病理生理学对于指导明确继发原因的诊断步骤很重要。应始终进行全面的补体分析，并伴有抗体筛查和遗传分析。尽管在临床试验中未得到系统验证，但已发表的证据为使用针对这些通常进展迅速且肾移植后常复发的疾病的现有治疗方法提供了坚实的基础。
