Anti-factor H autoantibody-associated hemolytic uremic syndrome: review of literature of the autoimmune form of HUS.

Abstract:

:Non-Shiga toxin-associated hemolytic uremic syndrome (atypical HUS) is a rare form of thrombotic microangiopathy that associates hemolytic anemia, thrombocytopenia, and acute renal failure. The disease has been demonstrated to be linked with a complement alternative pathway dysregulation due to genetic defects but also to development of autoantibodies to factor H (FH), the main plasmatic alternative pathway regulatory protein. In this review, we summarize the more recent data of this autoimmune form of HUS at the level of epidemiology and its clinical and biological features. We propose the performance of anti-FH autoantibodies screening at the very onset of the disease in all cases of HUS to first make the proper diagnosis as early as possible, and second to support an appropriate therapy including early plasma exchanges and immunosuppressive treatments.

journal_name

Semin Thromb Hemost

authors

Dragon-Durey MA,Blanc C,Garnier A,Hofer J,Sethi SK,Zimmerhackl LB

doi

10.1055/s-0030-1262885

subject

Has Abstract

pub_date

2010-09-01 00:00:00

pages

633-40

issue

6

eissn

0094-6176

issn

1098-9064

journal_volume

36

pub_type

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