Complement activation is involved in renal damage in human antineutrophil cytoplasmic autoantibody associated pauci-immune vasculitis

J Clin Immunol. 2009 May;29(3):282-91. doi: 10.1007/s10875-008-9268-2. Epub 2008 Dec 10.


Objective: This study was to investigate the evidence for complement activation in renal biopsy specimens of patients with myeloperoxidase (MPO)-antineutrophil cytoplasmic autoantibody (ANCA)-associated pauci-immune vasculitis.

Methods: Renal biopsy specimens from seven patients with MPO-ANCA positive pauci-immune necrotizing crescentic glomerulonephritis (NCGN) were used to detect the staining of membrane attack complex (MAC), C3d, C4d, mannose-binding lectin (MBL), factor B and factor P using immunohistochemistry and immunofluorescence. Renal tissue from seven patients with minimal change disease (MCD) and two normal renal tissue were used as controls.

Results: MAC, C3d, factor B and factor P could be detected in glomeruli and small blood vessels with active vasculitis of patients with pauci-immune AAV, but not or scarcely in patients with MCD and in normal renal tissue. C3d and factor B co-localized with MAC, factor P colocalized with C3d. MBL and C4d were not detected in patients with AAV.

Conclusion: The alternative pathway of the complement system is involved in renal damage of human pauci-immune AAV.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Adult
  • Aged
  • Antibodies, Antineutrophil Cytoplasmic / blood*
  • Antibodies, Antineutrophil Cytoplasmic / immunology
  • Antibody-Dependent Cell Cytotoxicity
  • Autoantibodies / blood*
  • Autoimmune Diseases / blood
  • Autoimmune Diseases / immunology*
  • Autoimmune Diseases / physiopathology
  • Complement Activation*
  • Complement Factor B / immunology
  • Complement Factor B / metabolism
  • Complement Membrane Attack Complex / immunology
  • Complement Membrane Attack Complex / metabolism
  • Female
  • Humans
  • Immunohistochemistry
  • Kidney / immunology*
  • Kidney / pathology
  • Male
  • Mannose-Binding Lectin / immunology
  • Mannose-Binding Lectin / metabolism
  • Middle Aged
  • Peroxidase / immunology
  • Properdin / immunology
  • Properdin / metabolism
  • Vasculitis / blood
  • Vasculitis / immunology*
  • Vasculitis / physiopathology


  • Antibodies, Antineutrophil Cytoplasmic
  • Autoantibodies
  • Complement Membrane Attack Complex
  • Mannose-Binding Lectin
  • Properdin
  • Peroxidase
  • Complement Factor B