Complement Activation Is Involved in Renal Damage in Human Antineutrophil Cytoplasmic Autoantibody Associated Pauci-Immune Vasculitis

  • Guang-qun Xing
  • Min Chen
  • Gang Liu
  • Peter Heeringa
  • Jun-jun Zhang
  • Xin Zheng
  • Jie E
  • Cees G. M. Kallenberg
  • Ming-hui Zhao



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.


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.


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.


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


ANCA complement histopathology myeloperoxidase pauci-immune vasculitis 



antineutrophil cytoplasmic autoantibody


ANCA-associated vasculitis




mannose-binding lectin


membrane attack complex


minimal change disease


necrotizing crescentic glomerulonephritis



This study is supported by a grant from National Science Fund for Distinguished Young Scholars (No.30725034) and a grant of the Chinese 985 project (985-2-104-113). P. Heeringa is supported by a grant from the Dutch Organization for Scientific research (NWO VIDI 917.66.342) and by a grant from the Dutch Kidney Foundation (PC 07-2204).

We are very grateful to Dr. Wan-zhong Zou and Dr. Chen Wang for assistance in the scoring and collecting pathological data of the patients.


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Copyright information

© Springer Science+Business Media, LLC 2008

Authors and Affiliations

  • Guang-qun Xing
    • 1
  • Min Chen
    • 1
  • Gang Liu
    • 1
  • Peter Heeringa
    • 3
  • Jun-jun Zhang
    • 1
  • Xin Zheng
    • 1
  • Jie E
    • 1
  • Cees G. M. Kallenberg
    • 2
  • Ming-hui Zhao
    • 1
  1. 1.Renal Division, Department of Medicine, Peking University First Hospital, Institute of Nephrology, Peking University, Key Laboratory of Renal DiseaseMinistry of Health of ChinaBeijingChina
  2. 2.Department of Clinical ImmunologyUniversity Medical Center GroningenGroningenThe Netherlands
  3. 3.Department of Pathology and Medical BiologyUniversity Medical Center GroningenGroningenThe Netherlands

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