ANCA-positive periaortic vasculitis: does it fall within the spectrum of vasculitis?

J Intern Med. 2002 Mar;251(3):268-71. doi: 10.1046/j.1365-2796.2002.00948.x.


Retroperitoneal fibrosis (RPF) is a disease of unknown aetiology that has sometimes been reported in association with connective tissue disorders and systemic vasculitis. We report here two cases of antineutrophil cytoplasmic antibody (ANCA)-positive RPF showing clinical evidence of rapidly progressive glomerulonephritis. Although treatment with prednisone and cyclophosphamide led to a remission of RPF in both cases, renal function was restored in only one patient and the other progressed to chronic renal failure. The paper reviews the literature concerning ANCA-positive RPF and discusses the relationship between ANCA-positive vasculitis and RPF.

Publication types

  • Case Reports

MeSH terms

  • Aged
  • Antibodies, Antineutrophil Cytoplasmic*
  • Disease Progression
  • Female
  • Glomerulonephritis / complications
  • Glomerulonephritis / pathology
  • Humans
  • Kidney Glomerulus / pathology
  • Male
  • Middle Aged
  • Retroperitoneal Fibrosis / complications
  • Retroperitoneal Fibrosis / drug therapy
  • Retroperitoneal Fibrosis / immunology*
  • Retroperitoneal Fibrosis / pathology
  • Vasculitis / immunology*


  • Antibodies, Antineutrophil Cytoplasmic