Abstract
Non-Shiga-toxin-associated hemolytic uremic syndrome (atypical HUS) is a rare form of thrombotic microangiopathy which associates hemolytic anemia, thrombocytopenia, and acute renal failure. In 10 % of cases the disease is linked to presence of autoantibodies directed against Factor H (FH), the main plasmatic alternative complement pathway regulatory protein. Their presence induces an acquired functional FH deficiency. The anti-FH autoantibodies screening must be performed at the very onset of the disease in all cases of HUS, in order, first, to make the proper diagnosis as early as possible, and second to support an appropriate therapy including early plasma exchanges and immunosuppressive treatments. Thus, anti-CFH IgG represents a diagnostic marker and the titer determination is useful for assessing disease evolution, because changes precede clinical symptoms, and for monitoring of treatment.
Presence of anti-FH IgG has been recently reported to be associated with other clinical context such as C3 glomerulopathies, but their pathogenicity in these conditions remains to be assessed.
Here we describe the ELISA assay allowing the detection of these autoantibodies and report the analysis which can be performed concomitantly to improve the diagnosis.
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Acknowledgement
This work was supported in part by grants from the Direction de la Recherche Clinique (DRC) of the Assistance Publique-Hôpitaux de Paris (APHP) (PHRC AOM05130; AOM08198; CIRC 06037) and by grants from Agence Nationale de la Recherche (ANR Genopath 2009–2012, 09geno031011).
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Dragon-Durey, MA. et al. (2014). Anti-Factor H Autoantibodies Assay. In: Gadjeva, M. (eds) The Complement System. Methods in Molecular Biology, vol 1100. Humana Press, Totowa, NJ. https://doi.org/10.1007/978-1-62703-724-2_20
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DOI: https://doi.org/10.1007/978-1-62703-724-2_20
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