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Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference

Timothy H.J. Goodship, H. Terence Cook, Fádi Fakhouri, Fernando C. Fervenza, Véronique Frémeaux‐Bacchi, David Kavanagh, Carla Nester, Marina Noris, Matthew C. Pickering, Santiago Rodrı́guez de Córdoba,

Kidney International · 2016 · 695 citations · 95 references

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TL;DR

Complement plays a primary role in the pathogenesis of both atypical hemolytic uremic syndrome and C3 glomerulopathy. The study reports the outcomes of a 2015 KDIGO Controversies Conference and discusses evidence‑based recommendations for treating aHUS and C3G. The conference addressed renal pathology, clinical phenotype, genetic and acquired disease drivers, and treatment strategies. Knowledge gaps were identified and a prioritized research agenda was proposed to resolve outstanding controversies.

Abstract

In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) complement plays a primary role in disease pathogenesis. Herein we report the outcome of a 2015 Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference where key issues in the management of these 2 diseases were considered by a global panel of experts. Areas addressed included renal pathology, clinical phenotype and assessment, genetic drivers of disease, acquired drivers of disease, and treatment strategies. In order to help guide clinicians who are caring for such patients, recommendations for best treatment strategies were discussed at length, providing the evidence base underpinning current treatment options. Knowledge gaps were identified and a prioritized research agenda was proposed to resolve outstanding controversial issues.