title: Outcome of renal transplantation in patients with pauci-immune small vessel vasculitis or anti-GBM disease.
abstract: OBJECTIVE
  Pauci-immune small vessel vasculitis (SVV) and anti-GBM disease are the most common causes of rapidly progressive glomerulonephritis (RPGN) and they frequently lead to end-stage renal disease. For renal replacement therapy, renal transplantation is the preferred treatment option. However, in patients with glomerular diseases, the outcome of renal transplantation can be adversely affected by recurrence of the original disease. The information in the medical literature on the outcome of renal transplantation in patients with RPGN is limited because most data are derived from case studies and from studies involving a small number of patients.
  
  
  METHODS
  We studied the outcome of renal transplantation in patients with pauciimmune SVV or anti-GBM disease, transplanted in our center between 1968 and 2000. Patient and graft survival were compared with a matched control group from our hospital. We specifically looked for any evidence of recurrent disease.
  
  
  RESULTS
  Included in the study were 43 patients (31 male, 12 female) with a mean age (+/- SD) of 48 +/- 15 years at transplantation. Patients were diagnosed as Wegener's granulomatosis (n = 8), microscopic polyangiitis (n = 7), renal limited vasculitis (n = 18) and anti-GBM disease (n = 10). The average follow-up was 62 +/- 57 months. No graft was lost due to recurrence of the underlying disease. One patient with Wegener's granulomatosis had a relapse with only extrarenal manifestations 5 months after transplantation. Patient and graft survival at 5 years after transplantation were 77% and 60%. Survival rates were not significantly different from a matched control group of renal transplant patients with other underlying diseases, 79% and 56%, respectively. Patients with pauci-immune SVV or anti-GBM disease developed significantly more malignancies than the control group (p = 0.02).
  
  
  CONCLUSIONS
  Recurrence of pauci-immune SVV and anti-GBM disease after transplantation is rare. Renal transplantation can be successfully performed in patients with pauciimmune vasculitis or anti-GBM disease. Physicians should be aware of the greater risk of developing malignancies, especially skin cancer.
authors.affiliation: Department of Medicine, Division of Nephrology, University Medical Center Nijmegen, Nijmegen, The Netherlands.
authors.forename: J K J
authors.initials: JK
authors.lastname: Deegens
authors.forename: M A
authors.initials: MA
authors.lastname: Artz
authors.forename: A J
authors.initials: AJ
authors.lastname: Hoitsma
authors.forename: J F M
authors.initials: JF
authors.lastname: Wetzels
mesh_terms: D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D019867:Anti-Glomerular Basement Membrane Disease / Q000150:complications* / Q000401:mortality / Q000601:surgery*; D005260:Female; D005921:Glomerulonephritis / Q000150:complications* / Q000401:mortality / Q000601:surgery*; D006085:Graft Survival; D006801:Humans; D007676:Kidney Failure, Chronic / Q000209:etiology* / Q000401:mortality / Q000601:surgery*; D016030:Kidney Transplantation; D008297:Male; D008875:Middle Aged; D017063:Outcome Assessment, Health Care; D012008:Recurrence; D012189:Retrospective Studies; D015996:Survival Rate; D014657:Vasculitis / Q000150:complications* / Q000401:mortality / Q000601:surgery*