Distribution of the granulocyte serine proteinases proteinase 3 and elastase in human glomerulonephritis

摘要: Abstract The serine proteinases proteinase 3 (PR3) and elastase are target antigens of antineutrophil cytoplasmic autoantibodies (ANCAs), which found in various systemic vasculitides with rapidly progressive glomerulonephritis (RPGN). expression both was studied immunohistologically (avidin-biotin complex method) murine monoclonal antibodies against PR3 (WGM2) (NP 57) 122 human renal biopsy specimens to investigate their role mediating damage. Expression predominated ANCA-associated RPGN independent the serologic ANCA pattern (c-/p-ANCA). staining patchy not always related distinct granulocytes due antigen spreading by disintegrating cells. It crescentic glomeruli interstitium ANCA-positive RPGN. In contrast, glomerular interstitial much more granulocyte even noncrescentic c-ANCA- p-ANCA-positive pauci-immune Endothelial cell basement membrane-bound or were observed. A faint PR3/elastase seen Goodpasture's syndrome within mesangioproliferative (granulocyte related). Both ANCA-negative acute postinfectious glomerulonephritis. conclusion, this study provides evidence, for first time, implication different forms proliferative suggests that finding is a marker neutrophil activation. circulating (ANCA) development characteristic vasculitis remains be elucidated.