Complement factor h-antibody-associated hemolytic uremic syndrome: pathogenesis, clinical presentation, and treatment.

Hofer, J. and Giner, T. and Józsi, Mihály (2014) Complement factor h-antibody-associated hemolytic uremic syndrome: pathogenesis, clinical presentation, and treatment. SEMINARS IN THROMBOSIS AND HEMOSTASIS, 40 (4). pp. 431-443. ISSN 0094-6176

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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.

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