Antiproteinase 3- and antimyeloperoxidase-associated vasculitis

Kidney Int. 2000 Jun;57(6):2195-206. doi: 10.1046/j.1523-1755.2000.00080.x.


Antiproteinase 3- and antimyeloperoxidase-associated vasculitis. Wegener's granulomatosis, microscopic polyangiitis, and idiopathic pauci-immune necrotizing crescentic glomerulonephritis (NCGN) are strongly associated with antineutrophil cytoplasmic autoantibodies (ANCAs) directed against either proteinase 3 (anti-PR3) or myeloperoxidase (anti-MPO). This has led some investigators to prefer combining these diseases under the common heading of ANCA-associated vasculitides. However, it is increasingly recognized that there are characteristic differences between patients with anti-PR3 and those with anti-MPO-associated vasculitis. This review focuses on the clinical, histopathologic, and possibly pathophysiologic differences between anti-PR3- and anti-MPO-associated vasculitis. Although there is considerable overlap, the anti-PR3- and anti-MPO-associated vasculitides are each characterized by particular clinical and histopathological findings. Extrarenal organ manifestations and respiratory tract granulomas occur more frequently in patients with anti-PR3 than in those with anti-MPO. Anti-PR3-positive patients with NCGN generally have a more dramatic deterioration of their renal function compared with anti-MPO-positive patients. The term "ANCA-associated vasculitis" is considered as a useful concept in the presence of systemic vasculitis. Likewise, in the presence of vasculitis, the terms "anti-PR3-associated vasculitis" and "anti-MPO-associated vasculitis" are useful concepts.

Publication types

  • Review

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic / analysis
  • Antibodies, Antineutrophil Cytoplasmic / immunology*
  • Humans
  • Kidney Diseases / immunology
  • Myeloblastin
  • Peroxidase / immunology*
  • Respiratory Tract Diseases / immunology
  • Serine Endopeptidases / immunology*
  • Vasculitis / immunology*
  • Vasculitis / physiopathology


  • Antibodies, Antineutrophil Cytoplasmic
  • Peroxidase
  • Serine Endopeptidases
  • Myeloblastin