H Terence Cook1. 1. Department of Medicine, Imperial College London, London, UK.
Abstract
PURPOSE OF REVIEW: The current review will discuss recent advances in our understanding of the pathology of C3 glomerulopathy and atypical haemolytic uremic syndrome (aHUS). RECENT FINDINGS: C3 glomerulopathy and aHUS are associated with abnormalities of control of the alternative pathway of complement. Recent articles have provided new insights into the classification of C3 glomerulopathy and its relationship to idiopathic immune complex-mediated glomerulonephritis. They suggest that there may be considerable overlap in pathogenesis between these entities and have indicated novel ways in which classification may be improved. There is increasing evidence that monoclonal gammopathy may cause C3 glomerulopathy or aHUS in older patients and emerging evidence that treatment of the underlying plasma cell clone may ameliorate the kidney disease. SUMMARY: Recent work has provided new insights into the causes of C3 glomerulopathy and aHUS, and the mechanism by which complement is dysregulated. This is of particular importance with the advent of new therapeutic agents which can specifically target different parts of the complement cascade.
PURPOSE OF REVIEW: The current review will discuss recent advances in our understanding of the pathology of C3 glomerulopathy and atypical haemolytic uremic syndrome (aHUS). RECENT FINDINGS:C3 glomerulopathy and aHUS are associated with abnormalities of control of the alternative pathway of complement. Recent articles have provided new insights into the classification of C3 glomerulopathy and its relationship to idiopathic immune complex-mediated glomerulonephritis. They suggest that there may be considerable overlap in pathogenesis between these entities and have indicated novel ways in which classification may be improved. There is increasing evidence that monoclonal gammopathy may cause C3 glomerulopathy or aHUS in older patients and emerging evidence that treatment of the underlying plasma cell clone may ameliorate the kidney disease. SUMMARY: Recent work has provided new insights into the causes of C3 glomerulopathy and aHUS, and the mechanism by which complement is dysregulated. This is of particular importance with the advent of new therapeutic agents which can specifically target different parts of the complement cascade.
Authors: Fei Zhao; Sara Afonso; Susanne Lindner; Andrea Hartmann; Ina Löschmann; Bo Nilsson; Kristina N Ekdahl; Lutz T Weber; Sandra Habbig; Gesa Schalk; Michael Kirschfink; Peter F Zipfel; Christine Skerka Journal: Front Immunol Date: 2019-05-31 Impact factor: 7.561
Authors: Fernando Caravaca-Fontán; Montserrat M Díaz-Encarnación; Laura Lucientes; Teresa Cavero; Virginia Cabello; Gema Ariceta; Luis F Quintana; Helena Marco; Xoana Barros; Natalia Ramos; Nuria Rodríguez-Mendiola; Sonia Cruz; Gema Fernández-Juárez; Adela Rodríguez; Ana Pérez de José; Cristina Rabasco; Raquel Rodado; Loreto Fernández; Vanessa Pérez Gómez; Ana I Ávila; Luis Bravo; Javier Lumbreras; Natalia Allende; Maria Dolores Sanchez de la Nieta; Eva Rodríguez; Teresa Olea; Marta Melgosa; Ana Huerta; Rosa Miquel; Carmen Mon; Gloria Fraga; Alberto de Lorenzo; Juliana Draibe; Marta Cano-Megías; Fayna González; Amir Shabaka; Maria Esperanza López-Rubio; María Ángeles Fenollosa; Luis Martín-Penagos; Iara Da Silva; Juana Alonso Titos; Santiago Rodríguez de Córdoba; Elena Goicoechea de Jorge; Manuel Praga Journal: Clin J Am Soc Nephrol Date: 2020-08-19 Impact factor: 8.237