Publication

Mechanisms of vasculitis: How pauci-immune is ANCA-associated renal vasculitis?

van Paassen, P., Tervaert, J. W. C. & Heeringa, P., 2007, In : Nephron experimental nephrology. 105, 1, p. E10-E16 7 p.

Research output: Contribution to journalReview articleAcademicpeer-review

Both the innate and the acquired immune system are involved in the pathophysiology of renal vasculitis. However, anti-neutrophil cytoplasmic antibody (ANCA)-associated renal vasculitis is characterized by a 'pauci-immune' pattern of immunofluorescence during kidney biopsy, indicating the relative lack of immunoglobulin and complement deposition within the kidney. On the other hand, evidence is accumulating that ANCA, autoantibodies against constituents of primary granules of neutrophils and the lysosomes of monocytes, play a pathogenic role in renal vasculitis. In this review we will discuss both in vitro and in vivo experimental data providing compelling evidence that ANCA are a primary pathogenic factor in renal vasculitis, mainly by augmenting leukocyte-endothelial interactions. We will also address novel data, pointing at the role of, in addition to ANCA, nonspecific proinflammatory signals. Finally, we propose a working hypothesis of the pathogenesis of ANCA-associated renal vasculitis. Copyright (c) 2007 S. Karger AG, Basel.

Original languageEnglish
Pages (from-to)E10-E16
Number of pages7
JournalNephron experimental nephrology
Volume105
Issue number1
Publication statusPublished - 2007

    Keywords

  • vasculitis, renal vasculitis, ANCA-associated renal vasculitis, glomerulonephritis, anti-neutrophil cytoplasmic antibody, neutrophil, ANTINEUTROPHIL CYTOPLASMIC ANTIBODIES, WEGENERS-GRANULOMATOSIS WG, NECROSIS-FACTOR-ALPHA, T-CELLS, CRESCENTIC GLOMERULONEPHRITIS, SYSTEMIC VASCULITIS, ENDOTHELIAL-CELLS, IN-VIVO, ANTIMYELOPEROXIDASE ANTIBODIES, ACTIVATE NEUTROPHILS

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