Atypical aHUS: State of the art

Carla M Nester; Thomas Barbour; Santiago Rodriquez de Cordoba; Marie Agnes Dragon-Durey; Veronique Fremeaux-Bacchi; Tim H J Goodship; David Kavanagh; Marina Noris; Matthew Pickering; Pilar Sanchez-Corral; Christine Skerka; Peter Zipfel; Richard J H Smith

doi:10.1016/j.molimm.2015.03.246

Atypical aHUS: State of the art

Mol Immunol. 2015 Sep;67(1):31-42. doi: 10.1016/j.molimm.2015.03.246. Epub 2015 Apr 3.

Affiliations

¹ Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, University of Iowa, Iowa City, IA, USA; Division of Nephrology, Stead Family Department of Pediatrics, Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, IA, USA.
² Centre for Complement and Inflammation Research, Department of Medicine, Imperial College, London W12 0NN, UK.
³ Centro Investigaciones Biológicas and Ciber de Enfermedades Raras, Ramiro de Maeztu 9, 28040 Madrid, Spain.
⁴ Assistance Publique - Hopitaux de Paris, Service d'Immunologie Biologique, Hopital Europeen Georges Pompidou, Paris, France.
⁵ Institute of Genetic Medicine, Newcastle University, Newcastle upon Tyne, UK.
⁶ IRCCS - Istituto di Ricerche Farmacologiche "Mario Negri", Clinical Research Center for Rare Diseases "Aldo e Cele Daccò", Ranica, Bergamo, Italy.
⁷ Unidad de Investigación and Ciber de Enfermedades Raras, Hospital Universitario de La Paz_IdiPAZ, Paseo de la Castellana 261, 28046 Madrid, Spain.
⁸ Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany.
⁹ Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany; Friedrich Schiller University, Jena, Germany.
¹⁰ Molecular Otolaryngology and Renal Research Laboratories, Carver College of Medicine, University of Iowa, Iowa City, IA, USA; Division of Nephrology, Stead Family Department of Pediatrics, Department of Internal Medicine, Carver College of Medicine, University of Iowa, Iowa City, IA, USA. Electronic address: richard-smith@uiowa.edu.

PMID: 25843230
DOI: 10.1016/j.molimm.2015.03.246

Abstract

Tremendous advances in our understanding of the thrombotic microangiopathies (TMAs) have revealed distinct disease mechanisms within this heterogeneous group of diseases. As a direct result of this knowledge, both children and adults with complement-mediated TMA now enjoy higher expectations for long-term health. In this update on atypical hemolytic uremic syndrome, we review the clinical characteristics; the genetic and acquired drivers of disease; the broad spectrum of environmental triggers; and current diagnosis and treatment options. Many questions remain to be addressed if additional improvements in patient care and outcome are to be achieved in the coming decade.

Publication types

Review

MeSH terms

Adult
Atypical Hemolytic Uremic Syndrome / etiology
Atypical Hemolytic Uremic Syndrome / genetics
Atypical Hemolytic Uremic Syndrome / immunology
Atypical Hemolytic Uremic Syndrome / pathology*
Autoantibodies / biosynthesis
Child
Communicable Diseases / complications
Communicable Diseases / genetics
Communicable Diseases / immunology
Communicable Diseases / pathology*
Complement Activation
Complement C3b / genetics
Complement C3b / immunology
Complement C3b Inactivator Proteins / genetics
Complement C3b Inactivator Proteins / immunology
Complement Factor H / genetics
Complement Factor H / immunology
Gene Expression Regulation / immunology*
Genetic Predisposition to Disease
Humans
Kidney / immunology
Kidney / pathology*
Kidney Neoplasms / complications
Kidney Neoplasms / genetics
Kidney Neoplasms / immunology
Kidney Neoplasms / pathology*
Kidney Transplantation

Substances

Autoantibodies
CFH protein, human
Complement C3b Inactivator Proteins
Complement C3b
Complement Factor H