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

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

Author information

1
Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK. Electronic address: tim.goodship@ncl.ac.uk.
2
Centre for Complement and Inflammation Research, Department of Medicine, Imperial College Hammersmith Campus, London, UK.
3
INSERM, UMR-S 1064, and Department of Nephrology and Immunology, CHU de Nantes, Nantes, France.
4
Department of Nephrology and Hypertension, Mayo Clinic, Rochester, Minnesota, USA.
5
Assistance Publique-Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Paris, France.
6
Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK.
7
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.
8
IRCCS-Istituto di Ricerche Farmacologiche "Mario Negri," Clinical Research Center for Rare Diseases "Aldo e Cele Daccò," Ranica, Bergamo, Italy.
9
Centro de Investigaciones Biológicas, Consejo Superior de Investigaciones Científicas, Madrid, Spain; Centro de Investigación Biomédica en Enfermedades Raras, Madrid, Spain.
10
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.
11
Department of Laboratory Medicine and Pathology, Mayo Clinic, Rochester, Minnesota, USA.
12
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.

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

PMID:
27989322
DOI:
10.1016/j.kint.2016.10.005
[Indexed for MEDLINE]
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