Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome

Mihály Józsi; Stefanie Reuter; Pilar Nozal; Margarita López-Trascasa; Pilar Sánchez-Corral; Zoltán Prohászka; Barbara Uzonyi

doi:10.1016/j.imlet.2014.01.014

Autoantibodies to complement components in C3 glomerulopathy and atypical hemolytic uremic syndrome

Immunol Lett. 2014 Aug;160(2):163-71. doi: 10.1016/j.imlet.2014.01.014. Epub 2014 Feb 1.

Authors

Mihály Józsi¹, Stefanie Reuter², Pilar Nozal³, Margarita López-Trascasa⁴, Pilar Sánchez-Corral⁵, Zoltán Prohászka⁶, Barbara Uzonyi⁷

Affiliations

¹ MTA-ELTE Lendület Complement Research Group, Department of Immunology, Eötvös Loránd University, Budapest, Hungary. Electronic address: [email protected].
² Junior Research Group for Cellular Immunobiology, Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany.
³ Unidad de Inmunología, Hospital Universitario La Paz, IdiPAZ, Madrid, Spain.
⁴ Unidad de Inmunología, Hospital Universitario La Paz, IdiPAZ, Madrid, Spain; CIBERER U754, Madrid, Spain.
⁵ CIBERER U754, Madrid, Spain; Unidad de Investigación, Hospital Universitario La Paz, IdiPAZ, Madrid, Spain.
⁶ Research Laboratory, 3rd Department of Internal Medicine, Semmelweis University, Budapest, Hungary.
⁷ MTA-ELTE Immunology Research Group, Department of Immunology, Eötvös Loránd University, Budapest, Hungary.

PMID: 24491679
DOI: 10.1016/j.imlet.2014.01.014

Abstract

The alternative pathway of complement is implicated in the pathogenesis of several renal diseases, such as atypical hemolytic uremic syndrome, dense deposit disease and other forms of C3 glomerulopathy. The underlying complement defects include genetic and/or acquired factors, the latter in the form of autoantibodies. Because the autoimmune forms require a specific treatment, in part different from that of the genetic forms, it is important to detect the autoantibodies as soon as possible and understand their characteristics. In this overview, we summarize the types of anti-complement autoantibodies detected in such diseases, i.e. autoantibodies to factor H, factor I, C3b, factor B and those against the C3 convertases (C3 nephritic factor and C4 nephritic factor). We draw attention to newly described autoantibodies and their characteristics, and highlight similarities and differences in the autoimmune forms of these diseases.

Keywords: Atypical hemolytic uremic syndrome; Autoantibodies; C3 convertase; C3 glomerulopathy; C3 nephritic factor; Complement regulation; Factor B; Factor H; Kidney disease.

Publication types

Research Support, Non-U.S. Gov't
Review

MeSH terms

Atypical Hemolytic Uremic Syndrome / genetics
Atypical Hemolytic Uremic Syndrome / immunology*
Atypical Hemolytic Uremic Syndrome / pathology
Autoantibodies / biosynthesis*
Autoantibodies / genetics
Autoantibodies / immunology
Complement C3-C5 Convertases / antagonists & inhibitors
Complement C3-C5 Convertases / genetics
Complement C3-C5 Convertases / immunology
Complement C3b / antagonists & inhibitors
Complement C3b / genetics
Complement C3b / immunology
Complement Factor B / antagonists & inhibitors
Complement Factor B / genetics
Complement Factor B / immunology
Complement Factor H / antagonists & inhibitors
Complement Factor H / genetics
Complement Factor H / immunology
Complement System Proteins / genetics
Complement System Proteins / immunology
Fibrinogen / antagonists & inhibitors
Fibrinogen / genetics
Fibrinogen / immunology
Gene Expression
Glomerulonephritis, IGA / genetics
Glomerulonephritis, IGA / immunology*
Glomerulonephritis, IGA / pathology
Humans

Substances

Autoantibodies
complement 4 nephritic factor
Complement C3b
Complement Factor H
Fibrinogen
Complement System Proteins
Complement C3-C5 Convertases
Complement Factor B