ABSTRACT
Atypical hemolytic uremic syndrome (aHUS) is a disease characterized by overactivation
of complement. Recurrence following renal transplantation is determined by a genetic
predisposition. Genetic screening of all individuals with aHUS should be performed
prior to listing for transplantation. Individuals with isolated mutations in MCP have a low risk of recurrence and may be considered for kidney transplantation alone.
In individuals with CFH and CFI mutations, the risk of recurrence following renal transplantation is high. Combined
liver/kidney transplantation has been used successfully in individuals with CFH mutations following the introduction of perioperative plasma exchange; however, such
a procedure is not without its risks. Liver/kidney transplantation has yet to be performed
on individuals with CFI and C3 mutations but may be predicted to be successful. In individuals with CFH autoantibodies, a reduction in titer through plasma exchange and rituximab has been
successful. Clinical trials of the complement C5 inhibitor eculizumab may improve
prospects for isolated renal transplantation in individuals with complement protein
mutations.
KEYWORDS
Transplantation - hemolytic uremic syndrome - complement - factor H - eculizumab
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David Kavanagh
Institute of Human Genetics, International Centre for Life, Central Parkway
Newcastle upon Tyne, NE1 3BZ United Kingdom
Email: davidkavanagh@doctors.org.uk
