Grimbert P, Schulte K, Buisson C, Desvaux D, Baron C, Pastural M, Dhamane D, Remy P, Weil B, Lang P
Department of Nephrology and Transplantation, Henri Mondor Teaching Hospital, Créteil, France.
Am J Kidney Dis. 2001 Jan;37(1):144-148. doi: 10.1016/s0272-6386(01)80068-8.
We describe a 36-year-old man who presented with hypocomplementemic urticarial vasculitis syndrome (HUVS) with severe renal involvement. Despite steroid therapy, the patient developed end-stage renal disease (ESRD) leading to chronic hemodialysis therapy. Renal transplantation was performed after hemodialysis therapy (secondary), and the patient developed a typical HUVS relapse 9 months after transplantation despite conventional immunosuppressive therapy that was successfully treated with plasma exchange. This case shows for the first time that HUVS can induce severe renal involvement responsible for ESRD and that HUVS can relapse after renal transplantation. It also suggests that plasma exchange therapy may be of value for rapidly controlling the clinical symptoms.
我们描述了一名36岁男性，他患有低补体血症性荨麻疹性血管炎综合征（HUVS）并伴有严重的肾脏受累。尽管接受了类固醇治疗，但患者仍发展为终末期肾病（ESRD），并导致了慢性血液透析治疗。在血液透析治疗（继发性）后进行了肾移植，尽管采用了常规免疫抑制治疗，但患者在移植后9个月出现了典型的HUVS复发，经血浆置换成功治疗。该病例首次表明，HUVS可导致严重的肾脏受累并引发ESRD，且肾移植后HUVS可能复发。这也提示血浆置换疗法可能对快速控制临床症状有价值。
