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. Bajema; Miguel 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

doi:10.1016/j.kint.2016.10.005

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 language	English (US)
Pages (from-to)	539-551
Number of pages	13
Journal	Kidney international
Volume	91
Issue number	3
DOIs	https://doi.org/10.1016/j.kint.2016.10.005
State	Published - Mar 1 2017

Keywords

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

ASJC Scopus subject areas

Nephrology

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10.1016/j.kint.2016.10.005

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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

Goodship, THJ, Cook, HT, Fakhouri, F, Fervenza, FC, Frémeaux-Bacchi, V, Kavanagh, D, Nester, CM, Noris, M, Pickering, MC, Rodríguez de Córdoba, S, Roumenina, LT, Sethi, S, Smith, RJH, Alpers, CE, Appel, GB, Ardissino, G, Ariceta, G, Arici, M, Bagga, A, Bajema, IM, Blasco, M, Burke, L, Cairns, TD, Carratala, M, D'Agati, VD, Daha, MR, De Vriese, AS, Dragon-Durey, MA, Fogo, AB, Galbusera, M, Gale, DP, Haller, H, Johnson, S, Józsi, M, Karpman, D, Lanning, L, Le Quintrec, M, Licht, C, Loirat, C, Monfort, F, Morgan, BP, Noël, LH, O'Shaughnessy, MM, Rabant, M, Rondeau, E, Ruggenenti, P, Sheerin, NS, Smith, J, Spoleti, F, Thurman, JM, van de Kar, NCAJ, Vivarelli, M & Zipfel, PF 2017, 'Atypical hemolytic uremic syndrome and C3 glomerulopathy: conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference', Kidney international, vol. 91, no. 3, pp. 539-551. https://doi.org/10.1016/j.kint.2016.10.005

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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.",

keywords = "C3 glomerulopathy, anti-complement therapies, atypical hemolytic uremic syndrome, complement, glomerulonephritis, kidney disease",

author = "Goodship, {Timothy H.J.} and Cook, {H. Terence} and Fadi Fakhouri and Fervenza, {Fernando C.} and V{\'e}ronique Fr{\'e}meaux-Bacchi and David Kavanagh and Nester, {Carla M.} and Marina Noris and Pickering, {Matthew C.} and {Rodr{\'i}guez de C{\'o}rdoba}, Santiago and Roumenina, {Lubka T.} and Sanjeev Sethi and Smith, {Richard J.H.} and Alpers, {Charlie E.} and Appel, {Gerald B.} and Gianluigi Ardissino and Gema Ariceta and Mustafa Arici and Arvind Bagga and Bajema, {Ingeborg M.} and Miguel Blasco and Linda Burke and Cairns, {Thomas D.} and Mireya Carratala and D'Agati, {Vivette D.} and Daha, {Mohamed R.} and {De Vriese}, {An S.} and Dragon-Durey, {Marie Agn{\`e}s} and Fogo, {Agnes B.} and Miriam Galbusera and Gale, {Daniel P.} and Hermann Haller and Sally Johnson and Mih{\'a}ly J{\'o}zsi and Diana Karpman and Lynne Lanning and {Le Quintrec}, Moglie and Christoph Licht and Chantal Loirat and Francisco Monfort and Morgan, {B. Paul} and No{\"e}l, {Laure H{\'e}l{\`e}ne} and O'Shaughnessy, {Michelle M.} and Marion Rabant and Eric Rondeau and Piero Ruggenenti and Sheerin, {Neil S.} and Jenna Smith and Fabrizio Spoleti and Thurman, {Joshua M.} and {van de Kar}, {Nicole C.A.J.} and Marina Vivarelli and Zipfel, {Peter F.}",

note = "Publisher Copyright: {\textcopyright} 2016 International Society of Nephrology",

year = "2017",

month = mar,

day = "1",

doi = "10.1016/j.kint.2016.10.005",

language = "English (US)",

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T2 - conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference

AU - Goodship, Timothy H.J.

AU - Cook, H. Terence

AU - Fakhouri, Fadi

AU - Fervenza, Fernando C.

AU - Frémeaux-Bacchi, Véronique

AU - Kavanagh, David

AU - Nester, Carla M.

AU - Noris, Marina

AU - Pickering, Matthew C.

AU - Rodríguez de Córdoba, Santiago

AU - Roumenina, Lubka T.

AU - Sethi, Sanjeev

AU - Smith, Richard J.H.

AU - Alpers, Charlie E.

AU - Appel, Gerald B.

AU - Ardissino, Gianluigi

AU - Ariceta, Gema

AU - Arici, Mustafa

AU - Bagga, Arvind

AU - Bajema, Ingeborg M.

AU - Blasco, Miguel

AU - Burke, Linda

AU - Cairns, Thomas D.

AU - Carratala, Mireya

AU - D'Agati, Vivette D.

AU - Daha, Mohamed R.

AU - De Vriese, An S.

AU - Dragon-Durey, Marie Agnès

AU - Fogo, Agnes B.

AU - Galbusera, Miriam

AU - Gale, Daniel P.

AU - Haller, Hermann

AU - Johnson, Sally

AU - Józsi, Mihály

AU - Karpman, Diana

AU - Lanning, Lynne

AU - Le Quintrec, Moglie

AU - Licht, Christoph

AU - Loirat, Chantal

AU - Monfort, Francisco

AU - Morgan, B. Paul

AU - Noël, Laure Hélène

AU - O'Shaughnessy, Michelle M.

AU - Rabant, Marion

AU - Rondeau, Eric

AU - Ruggenenti, Piero

AU - Sheerin, Neil S.

AU - Smith, Jenna

AU - Spoleti, Fabrizio

AU - Thurman, Joshua M.

AU - van de Kar, Nicole C.A.J.

AU - Vivarelli, Marina

AU - Zipfel, Peter F.

PY - 2017/3/1

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N2 - 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.

AB - 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.

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KW - anti-complement therapies

KW - atypical hemolytic uremic syndrome

KW - complement

KW - glomerulonephritis

KW - kidney disease

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

Abstract

Keywords

ASJC Scopus subject areas

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