Membrane surface proteinase 3 expression and intracytoplasmic immunoglobulin on neutrophils from patients with ANCA-associated vasculitides

Adv Exp Med Biol. 1993;336:45-50. doi: 10.1007/978-1-4757-9182-2_5.


We studied the presence of proteinase 3 (PR3), myeloperoxidase (MPO) and elastase (HLE) on the plasma membrane of neutrophils in patients with biopsy-proven Wegener's disease (WG), pANCA-positive vasculitis, control patients (SLE, rheumatoid arthritis, ankylosing spondylitis), sepsis patients and healthy donors. We found an overexpression of PR3 on the cell surface of neutrophils in WG, ANCA-associated vasculitis and during infection (sepsis). Thus PR3 becomes accessible to ANCA. Furthermore we detected intracytoplasmic IgG antibodies in PMN from patients with WG by immunoelectron microscopy and direct immunofluorescence. Our findings support the pathophysiological role of ANCA.

MeSH terms

  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies / blood*
  • Cell Membrane / enzymology
  • Cell Membrane / immunology
  • Granulomatosis with Polyangiitis / enzymology
  • Granulomatosis with Polyangiitis / immunology
  • Humans
  • Immunoglobulin G / blood*
  • Myeloblastin
  • Neutrophils / enzymology*
  • Neutrophils / immunology*
  • Pancreatic Elastase / blood
  • Peroxidase / blood
  • Serine Endopeptidases / blood*
  • Vasculitis / enzymology
  • Vasculitis / immunology*


  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies
  • Immunoglobulin G
  • Peroxidase
  • Serine Endopeptidases
  • Pancreatic Elastase
  • Myeloblastin