| Theme |
Designated intractable kidney diseases：The control in non-dialysis CKD and renal replacement therapy |
| Title |
Atypical hemolytic uremic syndrome |
| Author |
Madoka Fujisawa |
Division of Nephrology and Endocrinology, University of Tokyo |
| Author |
Hideki Kato |
Division of Nephrology and Endocrinology, University of Tokyo |
| Author |
Yoko Yoshida |
Division of Nephrology and Endocrinology, University of Tokyo |
| Author |
Masaomi Nangaku |
Division of Nephrology and Endocrinology, University of Tokyo |
| [ Summary ] |
Atypical hemolytic uremic syndrome (aHUS) is a severe kidney disease characterized by thrombocytopenia, hemolytic anemia, and acute renal injury. Recent studies have revealed that it is associated with abnormalities in genes encoding complement regulators, which lead to dysregulation of complement activation. These findings made it clear that eculizmab, the anti-C5 agent, would be a logical therapy, in conjunction with plasma therapy. A prospective two year follow up report revealed that eculizmab continues to be effective at blocking terminal complement and sustaining remission, and also is well tolerated. Despite this report, many questions concerning the use of eculizumab remain unresolved, requiring further trials. In addition, the importance of genetic screening is becoming greater, since outcomes vary according to the underlying complement alteration. |