title: Complement activation is involved in renal damage in human antineutrophil cytoplasmic autoantibody associated pauci-immune vasculitis.
abstract: 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.
  
  
  CONCLUSIONS
  The alternative pathway of the complement system is involved in renal damage of human pauci-immune AAV.
authors.affiliation: Renal Division, Department of Medicine, Peking University First Hospital, Institute of Nephrology, Peking University, Key Laboratory of Renal Disease, Ministry of Health of China, Beijing 100034, China.
authors.forename: Guang-qun
authors.initials: GQ
authors.lastname: Xing
authors.forename: Min
authors.initials: M
authors.lastname: Chen
authors.forename: Gang
authors.initials: G
authors.lastname: Liu
authors.forename: Peter
authors.initials: P
authors.lastname: Heeringa
authors.forename: Jun-jun
authors.initials: JJ
authors.lastname: Zhang
authors.forename: Xin
authors.initials: X
authors.lastname: Zheng
authors.forename: Jie
authors.initials: J
authors.lastname: E
authors.forename: Cees G M
authors.initials: CG
authors.lastname: Kallenberg
authors.forename: Ming-hui
authors.initials: MH
authors.lastname: Zhao
mesh_terms: D000293:Adolescent; D000328:Adult; D000368:Aged; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood* / Q000276:immunology; D000920:Antibody-Dependent Cell Cytotoxicity; D001323:Autoantibodies / Q000097:blood*; D001327:Autoimmune Diseases / Q000097:blood / Q000276:immunology* / Q000503:physiopathology; D003167:Complement Activation; D011415:Complement Factor B / Q000276:immunology / Q000378:metabolism; D015938:Complement Membrane Attack Complex / Q000276:immunology / Q000378:metabolism; D005260:Female; D006801:Humans; D007150:Immunohistochemistry; D007668:Kidney / Q000276:immunology* / Q000473:pathology; D008297:Male; D037601:Mannose-Binding Lectin / Q000276:immunology / Q000378:metabolism; D008875:Middle Aged; D009195:Peroxidase / Q000276:immunology; D011414:Properdin / Q000276:immunology / Q000378:metabolism; D014657:Vasculitis / Q000097:blood / Q000276:immunology* / Q000503:physiopathology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; D015938:Complement Membrane Attack Complex; D037601:Mannose-Binding Lectin; D011414:Properdin; D009195:Peroxidase; D011415:Complement Factor B