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, Fadi Fakhouri, Fernando C. Fervenza, Véronique Frémeaux-Bacchi, David Kavanagh, Carla M. Nester, Marina Noris, Matthew C. Pickering, Santiago Rodríguez de Córdoba, Lubka T. Roumenina, Sanjeev Sethi, Richard J.H. Smith, Charlie E. Alpers, Gerald B. Appel, Gianluigi Ardissino, Gema Ariceta, Mustafa Arici, Arvind Bagga, Ingeborg M. BajemaMiguel Blasco, Linda Burke, Thomas D. Cairns, Mireya Carratala, Vivette D. D'Agati, Mohamed R. Daha, An S. De Vriese, Marie Agnès Dragon-Durey, Agnes B. Fogo, Miriam Galbusera, Daniel P. Gale, Hermann Haller, Sally Johnson, Mihály Józsi, Diana Karpman, Lynne Lanning, Moglie Le Quintrec, Christoph Licht, Chantal Loirat, Francisco Monfort, B. Paul Morgan, Laure Hélène Noël, Michelle M. O'Shaughnessy, Marion Rabant, Eric Rondeau, Piero Ruggenenti, Neil S. Sheerin, Jenna Smith, Fabrizio Spoleti, Joshua M. Thurman, Nicole C.A.J. van de Kar, Marina Vivarelli, Peter F. Zipfel

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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.

Original languageEnglish (US)
Pages (from-to)539-551
Number of pages13
JournalKidney international
Volume91
Issue number3
DOIs
StatePublished - Mar 1 2017

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Keywords

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

ASJC Scopus subject areas

  • Nephrology

Cite this

Goodship, T. H. J., Cook, H. T., Fakhouri, F., Fervenza, F. C., Frémeaux-Bacchi, V., Kavanagh, D., Nester, C. M., Noris, M., Pickering, M. C., Rodríguez de Córdoba, S., Roumenina, L. T., Sethi, S., Smith, R. J. H., Alpers, C. E., Appel, G. B., Ardissino, G., Ariceta, G., Arici, M., Bagga, A., ... Zipfel, P. F. (2017). Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference. Kidney international, 91(3), 539-551. https://doi.org/10.1016/j.kint.2016.10.005