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

Research output: Contribution to journalArticle

121 Citations (Scopus)

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
Pages (from-to)539-551
Number of pages13
JournalKidney International
Volume91
Issue number3
DOIs
Publication statusPublished - Mar 1 2017

Fingerprint

Kidney Diseases
Inborn Genetic Diseases
Complement C3
Clinical Pathology
Disease Management
Therapeutics
Phenotype
Kidney
Research
Atypical Hemolytic Uremic Syndrome

Keywords

  • anti-complement therapies
  • atypical hemolytic uremic syndrome
  • C3 glomerulopathy
  • 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., ... 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

Atypical hemolytic uremic syndrome and C3 glomerulopathy : conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference. / Goodship, Timothy H.J.; Cook, H. Terence; Fakhouri, Fadi; Fervenza, Fernando C.; Frémeaux-Bacchi, Véronique; Kavanagh, David; Nester, Carla M.; Noris, Marina; Pickering, Matthew C.; Rodríguez de Córdoba, Santiago; Roumenina, Lubka T.; Sethi, Sanjeev; Smith, Richard J.H.; Alpers, Charlie E.; Appel, Gerald B.; Ardissino, Gianluigi; Ariceta, Gema; Arici, Mustafa; Bagga, Arvind; Bajema, Ingeborg M.; Blasco, Miguel; Burke, Linda; Cairns, Thomas D.; Carratala, Mireya; D'Agati, Vivette D.; Daha, Mohamed R.; De Vriese, An S.; Dragon-Durey, Marie Agnès; Fogo, Agnes B.; Galbusera, Miriam; Gale, Daniel P.; Haller, Hermann; Johnson, Sally; Józsi, Mihály; Karpman, Diana; Lanning, Lynne; Le Quintrec, Moglie; Licht, Christoph; Loirat, Chantal; Monfort, Francisco; Morgan, B. Paul; Noël, Laure Hélène; O'Shaughnessy, Michelle M.; Rabant, Marion; Rondeau, Eric; Ruggenenti, Piero; Sheerin, Neil S.; Smith, Jenna; Spoleti, Fabrizio; Thurman, Joshua M.; van de Kar, Nicole C.A.J.; Vivarelli, Marina; Zipfel, Peter F.

In: Kidney International, Vol. 91, No. 3, 01.03.2017, p. 539-551.

Research output: Contribution to journalArticle

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
Goodship, Timothy H.J. ; Cook, H. Terence ; Fakhouri, Fadi ; Fervenza, Fernando C. ; Frémeaux-Bacchi, Véronique ; Kavanagh, David ; Nester, Carla M. ; Noris, Marina ; Pickering, Matthew C. ; Rodríguez de Córdoba, Santiago ; Roumenina, Lubka T. ; Sethi, Sanjeev ; Smith, Richard J.H. ; Alpers, Charlie E. ; Appel, Gerald B. ; Ardissino, Gianluigi ; Ariceta, Gema ; Arici, Mustafa ; Bagga, Arvind ; Bajema, Ingeborg M. ; Blasco, Miguel ; Burke, Linda ; Cairns, Thomas D. ; Carratala, Mireya ; D'Agati, Vivette D. ; Daha, Mohamed R. ; De Vriese, An S. ; Dragon-Durey, Marie Agnès ; Fogo, Agnes B. ; Galbusera, Miriam ; Gale, Daniel P. ; Haller, Hermann ; Johnson, Sally ; Józsi, Mihály ; Karpman, Diana ; Lanning, Lynne ; Le Quintrec, Moglie ; Licht, Christoph ; Loirat, Chantal ; Monfort, Francisco ; Morgan, B. Paul ; Noël, Laure Hélène ; O'Shaughnessy, Michelle M. ; Rabant, Marion ; Rondeau, Eric ; Ruggenenti, Piero ; Sheerin, Neil S. ; Smith, Jenna ; Spoleti, Fabrizio ; Thurman, Joshua M. ; van de Kar, Nicole C.A.J. ; Vivarelli, Marina ; Zipfel, Peter F. / Atypical hemolytic uremic syndrome and C3 glomerulopathy : conclusions from a “Kidney Disease: Improving Global Outcomes” (KDIGO) Controversies Conference. In: Kidney International. 2017 ; Vol. 91, No. 3. pp. 539-551.
@article{8a18cc526c3a49048e5f2cc1da44b835,
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 = "anti-complement therapies, atypical hemolytic uremic syndrome, C3 glomerulopathy, 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.}",
year = "2017",
month = "3",
day = "1",
doi = "10.1016/j.kint.2016.10.005",
language = "English",
volume = "91",
pages = "539--551",
journal = "Kidney International",
issn = "0085-2538",
publisher = "Nature Publishing Group",
number = "3",

}

TY - JOUR

T1 - Atypical hemolytic uremic syndrome and C3 glomerulopathy

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

Y1 - 2017/3/1

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.

KW - anti-complement therapies

KW - atypical hemolytic uremic syndrome

KW - C3 glomerulopathy

KW - complement

KW - glomerulonephritis

KW - kidney disease

UR - http://www.scopus.com/inward/record.url?scp=85007574208&partnerID=8YFLogxK

UR - http://www.scopus.com/inward/citedby.url?scp=85007574208&partnerID=8YFLogxK

U2 - 10.1016/j.kint.2016.10.005

DO - 10.1016/j.kint.2016.10.005

M3 - Article

C2 - 27989322

AN - SCOPUS:85007574208

VL - 91

SP - 539

EP - 551

JO - Kidney International

JF - Kidney International

SN - 0085-2538

IS - 3

ER -