Outcomes of renal transplant in patients with anti-complement factor H antibody-associated hemolytic uremic syndrome.

Atypical HUS associated with anti-CFH autoantibodies is an uncommon illness associated with high risk of progression to end-stage renal disease. Disease relapses after transplantation, observed in one-third cases, often lead to graft loss. We report four patients with anti-CFH antibody-associated HUS who underwent renal transplantation 16-62 months from initial presentation. Two patients each received organs from deceased and living-related donors. Anti-CFH antibody titers were monitored during the illness and following transplantation. All patients received two doses of IV rituximab before or after transplantation; three patient each received 1-2 g/kg of IV immunoglobulin or underwent 2-5 sessions of plasma exchanges. The use of therapeutic plasma exchange, IV immunoglobulin, and rituximab in two cases enabled two-third reduction in anti-CFH antibody titers before transplantation. At 5- to 26-month follow-up, all patients showed satisfactory graft function without recurrence of HUS. This is the first report of patients with anti-CFH antibody-associated HUS who underwent living-related renal transplantation. Clearance of anti-CFH antibody by therapeutic plasma exchange and adjuvant immunosuppression aimed at decreasing antibody levels may enable successful transplantation and recurrence-free survival.
(© 2014 John Wiley & Sons A/S. Published by John Wiley & Sons Ltd.)