Journal
AMERICAN JOURNAL OF KIDNEY DISEASES
Volume 47, Issue 4, Pages 680-682Publisher
W B SAUNDERS CO-ELSEVIER INC
DOI: 10.1053/j.ajkd.2006.01.009
Keywords
antineutrophil cytoplasmic antibody (ANCA); vasculitis; acquired hemophilia; rituximab; autoimmunity; Campath-1H; regulatory T cells
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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.
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