title: Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a "Kidney Disease: Improving Global Outcomes" (KDIGO) Controversies Conference.
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.
authors.affiliation: Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK. Electronic address: tim.goodship@ncl.ac.uk.
authors.forename: Timothy H J
authors.initials: TH
authors.lastname: Goodship
authors.affiliation: Centre for Complement and Inflammation Research, Department of Medicine, Imperial College Hammersmith Campus, London, UK.
authors.forename: H Terence
authors.initials: HT
authors.lastname: Cook
authors.affiliation: INSERM, UMR-S 1064, and Department of Nephrology and Immunology, CHU de Nantes, Nantes, France.
authors.forename: Fadi
authors.initials: F
authors.lastname: Fakhouri
authors.affiliation: Department of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Fernando C
authors.initials: FC
authors.lastname: Fervenza
authors.affiliation: Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France.
authors.forename: Véronique
authors.initials: V
authors.lastname: Frémeaux-Bacchi
authors.affiliation: Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK.
authors.forename: David
authors.initials: D
authors.lastname: Kavanagh
authors.affiliation: Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA; Division of Nephrology, Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA.
authors.forename: Carla M
authors.initials: CM
authors.lastname: Nester
authors.affiliation: IRCCS-Istituto di Ricerche Farmacologiche "Mario Negri," Clinical Research Center for Rare Diseases "Aldo e Cele Daccò," Ranica, Bergamo, Italy.
authors.forename: Marina
authors.initials: M
authors.lastname: Noris
authors.affiliation: Centre for Complement and Inflammation Research, Department of Medicine, Imperial College Hammersmith Campus, London, UK.
authors.forename: Matthew C
authors.initials: MC
authors.lastname: Pickering
authors.affiliation: Centro de Investigaciones Biológicas, Consejo Superior de Investigaciones Científicas, Madrid, Spain; Centro de Investigación Biomédica en Enfermedades Raras, Madrid, Spain.
authors.forename: Santiago
authors.initials: S
authors.lastname: Rodríguez de Córdoba
authors.affiliation: Institut National de la Santé et de la Recherche Médicale, Unité Mixte de Recherche S1138, Complément et Maladies, Centre de Recherche des Cordeliers, Paris, France; Université Paris Descartes Sorbonne Paris-Cité, Paris, France; Université Pierre et Marie Curie (UPMC-Paris-6), Paris, France.
authors.forename: Lubka T
authors.initials: LT
authors.lastname: Roumenina
authors.affiliation: Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
authors.forename: Sanjeev
authors.initials: S
authors.lastname: Sethi
authors.affiliation: Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA; Division of Nephrology, Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, Iowa, USA. Electronic address: richard-smith@uiowa.edu.
authors.forename: Richard J H
authors.initials: RJ
authors.lastname: Smith
mesh_terms: D000818:Animals; D065766:Atypical Hemolytic Uremic Syndrome / Q000188:drug therapy / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D003167:Complement Activation / Q000187:drug effects; D003176:Complement C3 / Q000235:genetics / Q000276:immunology*; D051056:Complement Inactivating Agents / Q000627:therapeutic use; D020022:Genetic Predisposition to Disease; D005921:Glomerulonephritis / Q000188:drug therapy / Q000235:genetics / Q000276:immunology* / Q000473:pathology; D006801:Humans; D007668:Kidney / Q000187:drug effects / Q000276:immunology* / Q000473:pathology; D010641:Phenotype; D012307:Risk Factors; D016896:Treatment Outcome
chemical_list: C038245:C3 protein, human; D003176:Complement C3; D051056:Complement Inactivating Agents
keywords: C3 glomerulopathy; anti-complement therapies; atypical hemolytic uremic syndrome; complement; glomerulonephritis; kidney disease