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Semin Thromb Hemost 2014; 40(04): 431-443
DOI: 10.1055/s-0034-1375297
Thieme Medical Publishers 333 Seventh Avenue, New York, NY 10001, USA.

Complement Factor H-Antibody–Associated Hemolytic Uremic Syndrome: Pathogenesis, Clinical Presentation, and Treatment

Johannes Hofer
1   Department of Pediatrics I, Innsbruck Medical University, Innsbruck, Austria
,
Thomas Giner
1   Department of Pediatrics I, Innsbruck Medical University, Innsbruck, Austria
,
Mihály Józsi
2   MTA-ELTE “Lendület” Complement Research Group, Department of Immunology, Eötvös Loránd University, Budapest, Hungary
› Author Affiliations
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Publication History

Publication Date:
05 May 2014 (online)

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Abstract

The presence of circulating autoantibodies, primarily to complement factor H antibodies (CFH-Abs) in plasma characterizes the autoimmune form of atypical hemolytic uremic syndrome (aHUS). This acquired form of aHUS defines a distinct subgroup of aHUS patients, which requires diagnostic and treatment approaches in part different from those of the genetically defined forms. The mechanisms leading to CFH-Ab production and disease onset are not completely understood, but CFH-Ab HUS seems to be secondary to a combination of genetic predisposition and environmental factors. Early diagnosis of this specific aHUS entity is important, as prompt induction of plasma exchange and concomitant immunosuppression leads to a favorable outcome. Nevertheless, information on clinical features and outcome in children is limited. Here, we review the literature on the biological and clinical features of CFH-Ab HUS and discuss therapeutic options.

Keywords

complement factor H - complement factor H antibodies - atypical hemolytic uremic syndrome - complement-related diseases - transplantation
 

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