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

作者：Jozsi Mihaly^*; Reuter Stefanie; Nozal Pilar; Lopez Trascasa Margarita; Sanchez Corral Pilar; Prohaszka Zoltan; Uzonyi Barbara

来源：Immunology Letters, 2014, 160(2): 163-171.

DOI：10.1016/j.imlet.2014.01.014

• Autoantibodies
• Kidney disease
• Atypical hemolytic uremic syndrome
• C3 glomerulopathy
• Complement regulation
• C3 convertase
• Factor B
• Factor H
• C3 nephritic factor

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.

• 出版日期2014-8