title: Outcomes of renal transplant in patients with anti-complement factor H antibody-associated hemolytic uremic syndrome.
abstract: 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.
authors.affiliation: Division of Nephrology, Department of Pediatrics, All India Institute of Medical Sciences, New Delhi, India.
authors.forename: Priyanka
authors.initials: P
authors.lastname: Khandelwal
authors.forename: Aditi
authors.initials: A
authors.lastname: Sinha
authors.forename: Pankaj
authors.initials: P
authors.lastname: Hari
authors.forename: Virinder K
authors.initials: VK
authors.lastname: Bansal
authors.forename: Amit K
authors.initials: AK
authors.lastname: Dinda
authors.forename: Arvind
authors.initials: A
authors.lastname: Bagga
mesh_terms: D000293:Adolescent; D058846:Antibodies, Monoclonal, Murine-Derived / Q000627:therapeutic use; D001323:Autoantibodies / Q000097:blood*; D002648:Child; D017242:Complement Factor H / Q000276:immunology*; D006463:Hemolytic-Uremic Syndrome / Q000276:immunology* / Q000628:therapy*; D006801:Humans; D016756:Immunoglobulins, Intravenous / Q000627:therapeutic use*; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016030:Kidney Transplantation; D008297:Male; D010951:Plasma Exchange / Q000379:methods; D012008:Recurrence; D051437:Renal Insufficiency / Q000601:surgery; D000069283:Rituximab; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D058846:Antibodies, Monoclonal, Murine-Derived; D001323:Autoantibodies; D016756:Immunoglobulins, Intravenous; D007166:Immunosuppressive Agents; D000069283:Rituximab; D017242:Complement Factor H
keywords: intravenous immunoglobulin; plasmapheresis; recurrent disease; rituximab