Anti-Factor H autoantibodies in a fifth renal transplant recipient with atypical hemolytic and uremic syndrome.
Hemolytic uremic syndrome (HUS) associated with anti-Factor H (anti-FH) autoantibodies is a recently described pathophysiological entity. Monitoring of anti-FH IgG titer may be a sensitive marker of disease activity and guide treatment to eliminate circulating anti-FH antibodies. We report here a case of atypical HUS (aHUS) in which anti-FH autoantibodies were detected during the course of a fifth kidney transplant, 30 years after the first flare of aHUS. This exceptional case suggests that early, specific management based on immunosuppressive therapy and plasma exchanges monitored by anti-FH IgG titer may result in long-term graft survival.
Université Paris Descartes, Service de Transplantation Rénale et Soins Intensifs, Hôpital Necker, APHP, Paris, France. moglie.lequintrec@nck.aphp.fr
M
M
Le Quintrec
J
J
Zuber
L-H
LH
Noel
E
E
Thervet
V
V
Frémeaux-Bacchi
P
P
Niaudet
P
P
Niauif
W-H
WH
Fridman
C
C
Legendre
M-A
MA
Dragon-Durey
D001323:Autoantibodies / Q000097:blood*; D001798:Blood Proteins / Q000235:genetics; D002648:Child; D003180:Complement C3b Inactivator Proteins / Q000235:genetics; D011415:Complement Factor B / Q000276:immunology; D017242:Complement Factor H / Q000276:immunology*; D005260:Female; D017353:Gene Deletion; D006463:Hemolytic-Uremic Syndrome / Q000145:classification / Q000276:immunology / Q000601:surgery*; D006801:Humans; D016030:Kidney Transplantation / Q000276:immunology*; D012008:Recurrence; D012086:Reoperation / Q000706:statistics & numerical data
D001323:Autoantibodies; D001798:Blood Proteins; C517621:CFHR1 protein, human; C105970:CFHR3 protein, human; D003180:Complement C3b Inactivator Proteins; D017242:Complement Factor H; D011415:Complement Factor B