We describe a 36-year-old man who presented with hypocomplementemic urticar
ial vasculitis syndrome (HUVS) with severe renal involvement. Despite stero
id therapy, the patient developed end-stage renal disease (ESRD) leading to
chronic hemodialysis therapy. Renal transplantation was performed after he
modialysis therapy (secondary), and the patient developed a typical HUVS re
lapse 9 months after transplantation despite conventional immunosuppressive
therapy that was successfully treated with plasma exchange. This case show
s for the first time that HUVS can induce severe renal involvement responsi
ble for ESRD and that HUVS can relapse after renal transplantation. It also
suggests that plasma exchange therapy may be of value for rapidly controll
ing the clinical symptoms. (C) 2001 by the National Kidney Foundation, Inc.