Improved outcome in anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis: a 30-year follow-up study

Marc Hilhorst, Benjamin Wilde, Pieter van Paassen, Bjorn Winkens, Peter van Breda Vriesman, Jan Willem Cohen Tervaert*

*Corresponding author for this work

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87 Citations (Web of Science)


Background. Anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis has a poor prognosis. In the current study, we assessed whether prognosis in these patients improved over the last three decades. Methods. In a large inception cohort, all consecutive patients with ANCA-associated glomerulonephritis were included between January 1979 and December 2009. Inclusion criteria were the presence of ANCA and the availability of a kidney biopsy. To assess renal and patient survival, patients were divided in three groups through time: 1979-89, 1990-2000 and 2001-09. Results. A total of 181 patients were included. One-, 5- and 10-year survival was 77, 66 and 49%, respectively. Survival within the time groups was significantly different, yielding a hazard ratio for death of 2.9 for 1990-2000 and 3.9 for 1979-89 compared with 2001-09 (P <0.001). Serum creatinine and active lesions as found in the kidney biopsy significantly decreased through the three decades. Conclusions. Both patient and renal survival in patients with ANCA-associated renal vasculitis have improved over the last three decades. We postulate that both earlier diagnosis and better therapeutic management of patients are responsible for this effect.
Original languageEnglish
Pages (from-to)373-379
JournalNephrology Dialysis Transplantation
Issue number2
Publication statusPublished - Feb 2013


  • ANCA
  • outcome
  • renal biopsy
  • survival
  • vasculitis

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