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

Kidney Int. 2017 Mar;91(3):539-551. doi: 10.1016/j.kint.2016.10.005. Epub 2016 Dec 16.

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.

Keywords: C3 glomerulopathy; anti-complement therapies; atypical hemolytic uremic syndrome; complement; glomerulonephritis; kidney disease.

Publication types

  • Congress

MeSH terms

  • Animals
  • Atypical Hemolytic Uremic Syndrome / drug therapy
  • Atypical Hemolytic Uremic Syndrome / genetics
  • Atypical Hemolytic Uremic Syndrome / immunology*
  • Atypical Hemolytic Uremic Syndrome / pathology
  • Complement Activation* / drug effects
  • Complement C3 / genetics
  • Complement C3 / immunology*
  • Complement Inactivating Agents / therapeutic use
  • Genetic Predisposition to Disease
  • Glomerulonephritis / drug therapy
  • Glomerulonephritis / genetics
  • Glomerulonephritis / immunology*
  • Glomerulonephritis / pathology
  • Humans
  • Kidney / drug effects
  • Kidney / immunology*
  • Kidney / pathology
  • Phenotype
  • Risk Factors
  • Treatment Outcome

Substances

  • C3 protein, human
  • Complement C3
  • Complement Inactivating Agents