Incidence and outcome of pauci-immune rapidly progressive glomerulonephritis in Wessex, UK: a 10-year retrospective study

Nephrol Dial Transplant. 2000 Oct;15(10):1593-9. doi: 10.1093/ndt/15.10.1593.


Background: The Wessex Renal Unit serves a large stable population (2.5 million). Pauci-immune rapidly progressive glomerulonephritis (RPGN) is a frequent cause of acute renal failure requiring admission to our unit ( approximately 8%). At a population level, little is known of the epidemiology and outcome of RPGN.

Methods: Between 1 April 1986 and 31 March 1996, 141 cases of biopsy proven pauci-immune RPGN were seen in the Wessex region. The records of 128 patients were reviewed. Median (range) follow-up was 1.8 (0.9-9.64) years from diagnosis.

Results: The incidence of 4 per million was stable throughout the period. No clustering was seen. The diagnosis was made (median, 25th, 75th centile) 78.5 (45, 166) days after symptom onset. Co-morbidity (mostly hypertension) was seen in 47% of patients. Other organs affected were lungs 63%, nose/sinuses 50%, joints 42%, muscle 33%, skin 22% and nervous system 14%. Anti-neutrophil cytoplasmic antibody (ANCA) was positive in 73%; cytoplasmic ANCA 34%, peri-nuclear ANCA 26% and undifferentiated 14%. Twenty-seven per cent tested ANCA negative. The differences between the groups were small; time to diagnosis was shorter in the ANCA negative (-ve) group (P=0.02) and there were more airway symptoms in the ANCA positive (+ve) group (P:<0.05). All biopsies demonstrated a necrotizing process; crescents were seen in 96% involving (mean+/-SD) 54+/-26% of the glomeruli. Creatinine concentration (mean+/-SD) at diagnosis was 806+/-540 micromol/l. Treatment followed established immunosuppressive regimens. Initial dialysis was required by 59%, 36% needing long-term dialysis. At 1 year 68% were alive. The need for dialysis (P=0.0004) and age (P=0. 004) were poor prognostic markers. Ten per cent were transplanted, graft survival was 90% at 1 year, no recurrence was seen.

Conclusions: This study, looking at a large cohort, has established the incidence and outcome of ANCA +ve and ANCA -ve RPGN in a defined stable population. It stresses the similarities between ANCA +ve and ANCA -ve cases and supports the notion that pauci-immune RPGN is part of a continuum of vasculitic illness. In this series transplantation is a safe option.

MeSH terms

  • Age Distribution
  • Aged
  • Antibodies, Antineutrophil Cytoplasmic / metabolism
  • Antigen-Antibody Complex / analysis*
  • Cohort Studies
  • Disease Progression
  • Female
  • Glomerulonephritis / epidemiology*
  • Glomerulonephritis / immunology*
  • Glomerulonephritis / physiopathology
  • Glomerulonephritis / therapy
  • Humans
  • Incidence
  • Kidney Transplantation
  • Male
  • Middle Aged
  • Prognosis
  • Retrospective Studies
  • Survival Analysis
  • Time Factors
  • Tissue Distribution
  • Treatment Outcome
  • United Kingdom


  • Antibodies, Antineutrophil Cytoplasmic
  • Antigen-Antibody Complex