Complement Activation Is Involved in Renal Damage in Human Antineutrophil Cytoplasmic Autoantibody Associated Pauci-Immune Vasculitis
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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.
KeywordsANCA complement histopathology myeloperoxidase pauci-immune vasculitis
antineutrophil cytoplasmic autoantibody
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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