1. Academic Validation
  2. Understanding the complement-mediated glomerular diseases: focus on membranoproliferative glomerulonephritis and C3 glomerulopathies

Understanding the complement-mediated glomerular diseases: focus on membranoproliferative glomerulonephritis and C3 glomerulopathies

  • APMIS. 2016 Sep;124(9):725-35. doi: 10.1111/apm.12566.
Sophia Lionaki 1 Hara Gakiopoulou 2 John N Boletis 1
Affiliations

Affiliations

  • 1 Nephrology Department, Laiko Hospital, Faculty of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
  • 2 Department of Pathology, Faculty of Medicine, National and Kapodistrian University of Athens, Athens, Greece.
Abstract

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.

Keywords

C3 glomerulopathies; Membranoproliferative glomerulonephritis; classification.

Figures