Anti-factor H autoantibody-associated hemolytic uremic syndrome: review of literature of the autoimmune form of HUS.
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.
Dragon-Durey
Marie-Agnès
MA
Service d'Immunologie Biologique, Hôpital Européen Georges Pompidou, Assistance Publique-Hôpitaux de Paris, Paris, France. marie-agnes.durey@egp.ap-hop-paris.fr
Blanc
Caroline
C
Garnier
Arnaud
A
Hofer
Johannes
J
Sethi
Sidharth Kumar
SK
Zimmerhackl
Lothar-Bernd
LB
D001323:Autoantibodies / Q000276:immunology*; D003180:Complement C3b Inactivator Proteins / Q000235:genetics; D017242:Complement Factor H / Q000276:immunology*; D017353:Gene Deletion; D006463:Hemolytic-Uremic Syndrome / Q000235:genetics / Q000276:immunology* / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D010951:Plasma Exchange
D001323:Autoantibodies; C517621:CFHR1 protein, human; D003180:Complement C3b Inactivator Proteins; D007166:Immunosuppressive Agents; D017242:Complement Factor H