Successful renal transplantation in factor H autoantibody associated HUS with CFHR1 and 3 deficiency and CFH variant G2850T.
Factor H (CFH) autoantibodies are associated with atypical hemolytic uremic syndrome (aHUS). Peritransplantation plasma exchange therapy and intensification of immunosuppression, with adjuvant use of anti-CD20 monoclonal antibodies has recently been advocated for cases of CFH-autoantibody associated aHUS. In this report, we describe successful deceased donor renal transplantation in a case of CFH-autoantibody associated aHUS with combined CFHR1 and 3 deficiency in addition to the CFH sequence variant, (cG2850T, pGln950His). CFH-autoantibodies were detected 2 weeks prior to transplantation. Disease recurrence was not observed using basiliximab, an IL2-receptor antagonist and high-dose corticosteroids with mycophenolate mofetil. Adjuvant therapies such as Rituximab nor intensification of plasma therapy were employed. Consequently, careful consideration needs to be given to the use of additional immunosuppression in certain cases of CFH-autoantibody associated aHUS. Serial measurement of CFH-autoantibodies is required in the immediate pre- and posttransplantation period to further clarify their role as a factor in the recurrence of aHUS posttransplantation. Furthermore, delineation of the functional significance of CFH-autoantibodies is warranted in individual cases.
Waters
A M
AM
Department of Nephro-Urology, Great Ormond Street Hospital for Children NHS Trust, and Faculty of Medicine, Imperial College, London, UK. a.waters@ich.ucl.ac.uk
Pappworth
I
I
Marchbank
K
K
Bockenhauer
D
D
Tullus
K
K
Pickering
M C
MC
Strain
L
L
Sebire
N
N
Shroff
R
R
Marks
S D
SD
Goodship
T H J
TH
Rees
L
L
D019943:Amino Acid Substitution; D001323:Autoantibodies / Q000097:blood*; D001798:Blood Proteins / Q000172:deficiency*; D002648:Child; D003180:Complement C3b Inactivator Proteins / Q000172:deficiency*; D017242:Complement Factor H / Q000235:genetics* / Q000276:immunology*; D005260:Female; D014644:Genetic Variation; D006463:Hemolytic-Uremic Syndrome / Q000097:blood / Q000235:genetics / Q000276:immunology* / Q000601:surgery*; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016030:Kidney Transplantation / Q000276:immunology / Q000473:pathology / Q000502:physiology; D020641:Polymorphism, Single Nucleotide
D001323:Autoantibodies; D001798:Blood Proteins; C517621:CFHR1 protein, human; C105970:CFHR3 protein, human; D003180:Complement C3b Inactivator Proteins; D007166:Immunosuppressive Agents; D017242:Complement Factor H