Anti-Factor H autoantibodies in a fifth renal transplant recipient with atypical hemolytic and uremic syndrome

Am J Transplant. 2009 May;9(5):1223-9. doi: 10.1111/j.1600-6143.2009.02586.x.

Abstract

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.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Autoantibodies / blood*
  • Blood Proteins / genetics
  • Child
  • Complement C3b Inactivator Proteins / genetics
  • Complement Factor B / immunology
  • Complement Factor H / immunology*
  • Female
  • Gene Deletion
  • Hemolytic-Uremic Syndrome / classification
  • Hemolytic-Uremic Syndrome / immunology
  • Hemolytic-Uremic Syndrome / surgery*
  • Humans
  • Kidney Transplantation / immunology*
  • Recurrence
  • Reoperation / statistics & numerical data

Substances

  • Autoantibodies
  • Blood Proteins
  • CFHR1 protein, human
  • CFHR3 protein, human
  • Complement C3b Inactivator Proteins
  • Complement Factor H
  • Complement Factor B