Acquired von Willebrand syndrome in systemic lupus erythematodes

Acquired von Willebrand syndrome (AvWS) in systemic lupus erythematodes (SL E) is caused by autoantibodies directed against the circulating von Willebr and factor (vWF)/factor VIII (FVIII) complex. The autoantibody-vWF/FVIII an tigen complex is cleared rapidly from the circulation, leading to a moderat e to severe quantitative and qualitative deficiency of both vWF and FVIIIc. Consequently, AvWS in SLE is featured by a prolonged bleeding time and nor mal platelet count, a prolonged activated partial thromboplastin time (APTT ) and normal prothrombin time (PT), decreased or absent ristocetin-induced platelet aggregation (RIPA), and type II vWF deficiency on multimeric analy sis of the vWF protein. Acquired von Willebrand syndrome type II in SLE res ponds poorly to 1-desamino-8-D-arginine vasopressin (DDAVP) and FVIII conce ntrate, but responds transiently well to high-dose gammaglobulin given intr avenously. All reported cases of AvWS in SLE were cured by appropriate trea tment of the underlying autoimmune disease with prednisone or immunosuppres sion.