Acquired hemophilia in association with ANCA-associated vasculitis: response to rituximab

Am J Kidney Dis. 2006 Apr;47(4):680-2. doi: 10.1053/j.ajkd.2006.01.009.


We describe a patient with end-stage renal disease secondary to antineutrophil cytoplasmic antibody-associated vasculitis who subsequently developed acquired hemophilia A with autoantibodies to factor VIII. This is a novel association. Previous vasculitis therapy with the anti-CD52 monoclonal antibody Campath-1H may have contributed to the development of a second autoimmune disease in this patient by inadvertent depletion of regulatory T cells. The hemophilia followed a relapsing course under oral corticosteroid therapy, but B-cell depletion with anti-CD20 monoclonal antibody (rituximab) was effective in inducing a prolonged remission.

Publication types

  • Case Reports

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic*
  • Antibodies, Monoclonal / therapeutic use*
  • Antibodies, Monoclonal, Murine-Derived
  • Hemophilia A / drug therapy*
  • Hemophilia A / etiology*
  • Humans
  • Immunologic Factors / therapeutic use*
  • Male
  • Middle Aged
  • Rituximab
  • Vasculitis / complications*
  • Vasculitis / immunology*


  • Antibodies, Antineutrophil Cytoplasmic
  • Antibodies, Monoclonal
  • Antibodies, Monoclonal, Murine-Derived
  • Immunologic Factors
  • Rituximab