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

被引：475

作者：

Goodship, Timothy H. J. ^{[1
]}

Cook, H. Terence ^{[2
]}

Fakhouri, Fadi ^{[3
,4
]}

Fervenza, Fernando C. ^{[5
]}

Fremeaux-Bacchi, Veronique ^{[6
]}

Kavanagh, David ^{[1
]}

Nester, Carla M. ^{[7
,8
]}

Noris, Marina ^{[9
]}

Pickering, Matthew C. ^{[2
]}

de Cordoba, Santiago Rodriguez ^{[10
,11
]}

Roumenina, Lubka T. ^{[12
,13
,14
]}

Sethi, Sanjeev ^{[15
]}

Smith, Richard J. H. ^{[7
,8
]}

机构：

[1] Newcastle Univ, Inst Med Genet, Newcastle Upon Tyne NE1 3BZ, Tyne & Wear, England

[2] Ctr Complement & Inflammat Res, Dept Med, Imperial Coll Hammersmith Campus, London, England

[3] CHU Nantes, INSERM, UMR S 1064, Nantes, France

[4] CHU Nantes, Dept Nephrol & Immunol, Nantes, France

[5] Mayo Clin, Dept Hypertens & Nephrol, Rochester, MN USA

[6] Hop Europeen Georges Pompidou, AP HP, Paris, France

[7] Univ Iowa, Carver Coll Med, Mol Otolaryngol & Renal Res Labs, Iowa City, IA USA

[8] Univ Iowa, Carver Coll Med, Dept Internal Med, Div Nephrol, Iowa City, IA USA

[9] IRCCS, Ist Ric Farmacol Mario Negri, Clin Res Ctr Rare Dis Aldo & Cele Dacco Ranica, Bergamo, Italy

[10] CSIC, Ctr Invest Biol, Madrid, Spain

[11] Ctr Invest Biomed Enfermedades Raras, Madrid, Spain

[12] Inst Natl Sante & Rech Med, Ctr Rech Cordeliers, Unite Mixte Rech 51138, Complement & Malad, Paris, France

[13] Univ Paris 05, Sorbonne Paris Cite, Paris, France

[14] UPMC Paris 6, Paris, France

[15] Mayo Clin, Dept Lab Med & Pathol, Rochester, MN USA

来源：

KIDNEY INTERNATIONAL | 2017年 / 91卷 / 03期

基金：

英国惠康基金; 英国医学研究理事会;

关键词：

anti-complement therapies; atypical hemolytic uremic syndrome; C3; glomerulopathy; complement; glomerulonephritis; kidney disease; COMPLEMENT FACTOR-H; DENSE-DEPOSIT DISEASE; ECULIZUMAB MAINTENANCE TREATMENT; MEMBRANOPROLIFERATIVE GLOMERULONEPHRITIS; MESANGIOCAPILLARY GLOMERULONEPHRITIS; RENAL-TRANSPLANTATION; OXFORD CLASSIFICATION; MACULAR DEGENERATION; ALTERNATIVE PATHWAY; CLINICAL-FEATURES;

D O I：

10.1016/j.kint.2016.10.005

中图分类号：

R5 [内科学]; R69 [泌尿科学（泌尿生殖系疾病）];

学科分类号：

1002 ; 100201 ;

摘要：

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.

引用

页码：539 / 551

页数：13

共 114 条

[1] Characterization of complement factor H-related (CFHR) proteins in plasma reveals novel genetic variations of CFHR1 associated with atypical hemolytic uremic syndrome
Abarrategui-Garrido, Cynthia
Martinez-Barricarte, Ruben
Lopez-Trascasa, Margarita
Rodriguez de Cordoba, Santiago
Sanchez-Corral, Pilar
[J]. BLOOD, 2009, 114 (19) : 4261 - 4271
[2] Allelic Variants of Complement Genes Associated with Dense Deposit Disease
Abrera-Abeleda, Maria Asuncion
Nishimura, Carla
Frees, Kathy
Jones, Michael
Maga, Tara
Katz, Louis M.
Zhang, Yuzhou
Smith, Richard J. H.
[J]. JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2011, 22 (08): : 1551 - 1559
[3] Complement Factor H Autoantibodies Are Associated with Early Stage NSCLC
Amornsiripanitch, Nita
Hong, Shaolin
Campa, Michael J.
Frank, Michael M.
Gottlin, Elizabeth B.
Patz, Edward F., Jr.
[J]. CLINICAL CANCER RESEARCH, 2010, 16 (12) : 3226 - 3231
[4] Diagnosis of complement alternative pathway disorders
Angioi, Andrea
Fervenza, Fernando C.
Sethi, Sanjeev
Zhang, Yuzhou
Smith, Richard J.
Murray, David
Van Praet, Jens
Pani, Antonello
De Vriese, An S.
[J]. KIDNEY INTERNATIONAL, 2016, 89 (02) : 278 - 288
[5] Discontinuation of Eculizumab Treatment in Atypical Hemolytic Uremic Syndrome: An Update
Ardissino, Gianluigi
Possenti, Ilaria
Tel, Francesca
Testa, Sara
Salardi, Stefania
Ladisa, Vito
[J]. AMERICAN JOURNAL OF KIDNEY DISEASES, 2015, 66 (01) : 172 - 173
[6] Discontinuation of Eculizumab Maintenance Treatment for Atypical Hemolytic Uremic Syndrome: A Report of 10 Cases
Ardissino, Gianluigi
Testa, Sara
Possenti, Ilaria
Tel, Francesca
Paglialonga, Fabio
Salardi, Stefania
Tedeschi, Silvana
Belingheri, Mirco
Cugno, Massimo
[J]. AMERICAN JOURNAL OF KIDNEY DISEASES, 2014, 64 (04) : 633 - 637
[7] Familial C3 Glomerulopathy Associated with CFHR5 Mutations: Clinical Characteristics of 91 Patients in 16 Pedigrees
Athanasiou, Yiannis
Voskarides, Konstantinos
Gale, Daniel P.
Damianou, Loukas
Patsias, Charalambos
Zavros, Michalis
Maxwell, Patrick H.
Cook, H. Terence
Demosthenous, Panayiota
Hadjisavvas, Andreas
Kyriacou, Kyriacos
Zouvani, Ioanna
Pierides, Alkis
Deltas, Constantinos
[J]. CLINICAL JOURNAL OF THE AMERICAN SOCIETY OF NEPHROLOGY, 2011, 6 (06): : 1436 - 1446
[8] Human factor H deficiency - Mutations in framework cysteine residues and block in H protein secretion and intracellular catabolism
Ault, BH
Schmidt, BZ
Fowler, NL
Kashtan, CE
Ahmed, AE
Vogt, BA
Colten, HR
[J]. JOURNAL OF BIOLOGICAL CHEMISTRY, 1997, 272 (40) : 25168 - 25175
[9] The MEST score provides earlier risk prediction in IgA nephropathy
Barbour, Sean J.
Espino-Hernandez, Gabriela
Reich, Heather N.
Coppo, Rosanna
Roberts, Ian S. D.
Feehally, John
Herzenberg, Andrew M.
Cattran, Daniel C.
[J]. KIDNEY INTERNATIONAL, 2016, 89 (01) : 167 - 175
[10] Update on C3 glomerulopathy
Barbour, Thomas D.
Ruseva, Marieta M.
Pickering, Matthew C.
[J]. NEPHROLOGY DIALYSIS TRANSPLANTATION, 2016, 31 (05) : 717 - 725

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