Acquired hemophilia in association with ANCA-associated vasculitis: response to rituximab.
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.
Clatworthy
Menna R
MR
Lupus and Vasculitis Clinic, Department of Medicine, University of Cambridge School of Clinical Medicine, Cambridge, UK. mrc38@cam.ac.uk
Jayne
David R W
DR
D019268:Antibodies, Antineutrophil Cytoplasmic; D000911:Antibodies, Monoclonal / Q000627:therapeutic use*; D058846:Antibodies, Monoclonal, Murine-Derived; D006467:Hemophilia A / Q000188:drug therapy* / Q000209:etiology*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use*; D008297:Male; D008875:Middle Aged; D000069283:Rituximab; D014657:Vasculitis / Q000150:complications* / Q000276:immunology*
D019268:Antibodies, Antineutrophil Cytoplasmic; D000911:Antibodies, Monoclonal; D058846:Antibodies, Monoclonal, Murine-Derived; D007155:Immunologic Factors; D000069283:Rituximab