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

Timothy Goodship; H Cook; Fadi Fakhouri; Fernando Fervenza; Véronique Frémeaux-Bacchi; David Kavanagh; Carla Nester; Marina Noris; Matthew Pickering; Santiago Rodríguez de Córdoba; Lubka Roumenina; Sanjeev Sethi; Richard Smith

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

Timothy Goodship ¹ H Cook ² Fadi Fakhouri ^{3, 4} Fernando Fervenza ⁵ Véronique Frémeaux-Bacchi ⁶ David Kavanagh ¹ Carla Nester ^{7, 8} Marina Noris ⁹ Matthew Pickering ² Santiago Rodríguez de Córdoba ¹⁰ Lubka Roumenina ^{11, 12, 13, 14} Sanjeev Sethi ¹⁵ Richard Smith ^{7, 8, *}

* Corresponding author

1 Institute of Genetic Medicine [Newcastle, U.K.]

2 Department of Medicine - Centre for Complement and Inflammation Research [London, UK]

3 U1064 Inserm - CRTI - Centre de Recherche en Transplantation et Immunologie

4 Département de néphrologie et d'immunologie [CHU Nantes]

5 Department of Nephrology and Hypertension [Rochester, MN, USA]

6 HEGP - Hôpital Européen Georges Pompidou [APHP]

7 Carver College of Medicine - Molecular Otolaryngology and Renal Research Laboratories [Iowa City, IA, USA]

8 Department of Internal Medicine [Iowa City]

9 RCCS–Istituto di Ricerche Farmacologiche “Mario Negri [Bergamo, Italy]

10 CSIC - Centro de Investigaciones Biológicas

11 CRC - U1138 - Centre de Recherche des Cordeliers

12 UPD5 - Université Paris Descartes - Paris 5

13 USPC - Université Sorbonne Paris Cité

14 UPMC - Université Pierre et Marie Curie - Paris 6

15 Department of Laboratory Medicine and Pathology (Mayo Clinic Rochester)

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

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Journal articles

Domain :

Life Sciences [q-bio] / Human health and pathology

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https://www.hal.inserm.fr/inserm-02155926
Contributor : Ana Paula Dutra Azevedo <>
Submitted on : Friday, June 14, 2019 - 8:54:11 AM
Last modification on : Friday, August 2, 2019 - 10:44:34 AM

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

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