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Semin Thromb Hemost 2010; 36(6): 633-640
DOI: 10.1055/s-0030-1262885
© Thieme Medical Publishers

Anti-Factor H Autoantibody–Associated Hemolytic Uremic Syndrome: Review of Literature of the Autoimmune Form of HUS

Marie-Agnès Dragon-Durey1 , 2 , Caroline Blanc2 , Arnaud Garnier3 , Johannes Hofer4 , Sidharth Kumar Sethi5 , Lothar-Bernd Zimmerhackl4
  • 1Service d'Immunologie Biologique, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France
  • 2Unité INSERM UMRS 872, Centre de Recherche des Cordeliers, Université Paris 5, Paris, France
  • 3Service de Néphrologie, Hôpital Robert Debré, Assistance Publique-Hôpitaux de Paris, Université Paris-Diderot, Paris, France
  • 4Department of Pediatrics, Pediatrics I, Medical University Innsbruck, Innsbruck, Austria
  • 5Division of Pediatric Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India
Further Information

Publication History

Publication Date:
23 September 2010 (online)

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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.

KEYWORDS

Atypical hemolytic uremic syndrome - complement factor H - autoantibodies - complement factor H–related proteins

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Dr. Marie-Agnès Dragon-Durey

Service d'Immunologie Biologique, Hôpital Européen Georges Pompidou

20 rue Leblanc, 75 015 Paris, France

Email: marie-agnes.durey@egp.ap-hop-paris.fr

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