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 ca...

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Veröffentlicht in:American journal of transplantation 2009-05, Vol.9 (5), p.1223-1229
Hauptverfasser: Le Quintrec, M., Zuber, J., Noel, L.‐H., Thervet, E., Frémeaux‐Bacchi, V., Fridman, W.‐H., Legendre, C., Dragon‐Durey, M.‐A.
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container_end_page 1229
container_issue 5
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container_title American journal of transplantation
container_volume 9
creator Le Quintrec, M.
Zuber, J.
Noel, L.‐H.
Thervet, E.
Frémeaux‐Bacchi, V.
Fridman, W.‐H.
Legendre, C.
Dragon‐Durey, M.‐A.
description 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. The detection and monitoring of anti Factor‐H auto‐antibodies in an atypical HUS case prevented a relapse in a fifth transplantation.
doi_str_mv 10.1111/j.1600-6143.2009.02586.x
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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. 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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. The detection and monitoring of anti Factor‐H auto‐antibodies in an atypical HUS case prevented a relapse in a fifth transplantation.</description><subject>Autoantibodies - blood</subject><subject>Biological and medical sciences</subject><subject>Blood Proteins - genetics</subject><subject>Child</subject><subject>Complement C3b Inactivator Proteins - genetics</subject><subject>Complement Factor B - immunology</subject><subject>Complement Factor H - immunology</subject><subject>Female</subject><subject>Gene Deletion</subject><subject>Hematologic and hematopoietic diseases</subject><subject>Hemolytic-Uremic Syndrome - classification</subject><subject>Hemolytic-Uremic Syndrome - immunology</subject><subject>Hemolytic-Uremic Syndrome - surgery</subject><subject>Humans</subject><subject>Kidney Transplantation - immunology</subject><subject>Medical sciences</subject><subject>Nephrology. Urinary tract diseases</subject><subject>Nephropathies. Renovascular diseases. Renal failure</subject><subject>Platelet diseases and coagulopathies</subject><subject>Recurrence</subject><subject>Renal failure</subject><subject>Reoperation - statistics &amp; numerical data</subject><subject>Surgery (general aspects). Transplantations, organ and tissue grafts. 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source MEDLINE; Wiley Journals; EZB-FREE-00999 freely available EZB journals; Alma/SFX Local Collection
subjects Autoantibodies - blood
Biological and medical sciences
Blood Proteins - genetics
Child
Complement C3b Inactivator Proteins - genetics
Complement Factor B - immunology
Complement Factor H - immunology
Female
Gene Deletion
Hematologic and hematopoietic diseases
Hemolytic-Uremic Syndrome - classification
Hemolytic-Uremic Syndrome - immunology
Hemolytic-Uremic Syndrome - surgery
Humans
Kidney Transplantation - immunology
Medical sciences
Nephrology. Urinary tract diseases
Nephropathies. Renovascular diseases. Renal failure
Platelet diseases and coagulopathies
Recurrence
Renal failure
Reoperation - statistics & numerical data
Surgery (general aspects). Transplantations, organ and tissue grafts. Graft diseases
title Anti‐Factor H Autoantibodies in a Fifth Renal Transplant Recipient with Atypical Hemolytic and Uremic Syndrome
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