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 Blood, 1 February 2008, Vol. 111, No. 3, pp. 1512-1514.
Prepublished online as a Blood First Edition Paper on November 15, 2007; DOI 10.1182/blood-2007-09-109876.

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IMMUNOBIOLOGY

Brief Report

Factor H autoantibodies in atypical hemolytic uremic syndrome correlate with CFHR1/CFHR3 deficiency

Mihály Józsi1, Christoph Licht2, Stefanie Strobel1, Svante L. H. Zipfel1, Heiko Richter1, Stefan Heinen1, Peter F. Zipfel1,3, and Christine Skerka1

1 Leibniz Institute for Natural Product Research and Infection Biology, Jena, Germany; 2 Division of Nephrology, Hospital for Sick Children, Toronto, ON; and 3 Friedrich Schiller University, Jena, Germany

Atypical hemolytic uremic syndrome (aHUS) is a severe renal disease that is associated with defective complement regulation caused by multiple factors. We previously described the deficiency of factor H–related proteins CFHR1 and CFHR3 as predisposing factor for aHUS. Here we identify in an extended cohort of 147 aHUS patients that 16 juvenile individuals (ie, 11%) who either lacked the CFHR1/CFHR3 completely (n = 14) or showed extremely low CFHR1/CFHR3 plasma levels (n = 2) are positive for factor H (CFH) autoantibodies. The binding epitopes of all 16 analyzed autoantibodies were localized to the C-terminal recognition region of factor H, which represents a hot spot for aHUS mutations. Thus we define a novel subgroup of aHUS, termed DEAP HUS (deficiency of CFHR proteins and CFH autoantibody positive) that is characterized by a combination of genetic and acquired factors. Screening for both factors is obviously relevant for HUS patients as reduction of CFH autoantibody levels represents a therapeutic option.


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