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Autoimmunity in kidney diseases

Item Type:Article
Title:Autoimmunity in kidney diseases
Creators Name:Kettritz, R.
Abstract:Renal involvement in autoimmunity has many facets. Glomerular, tubular and vascular structures are targeted and damaged as a consequence of autoimmune processes. Most dramatic and life-threatening causes are observed with diseases that result in rapidly progressive glomerulonephritis (GN), frequently accompanied by involvement of additional non-renal organs. Typical diseases with these characteristics are anti-neutrophil cytoplasmic antibody (ANCA)-associated vasculitidis, anti-glomerular basement membrane (anti-GBM) GN and proliferative lupus nephritis. The leading cause of rapidly progressive GN is ANCA-associated GN and is the main focus of this article. Two major ANCA antigens have been described, namely proteinase 3 (PR3), with a cytoplasmic immunfluorescence staining pattern, and myeloperoxidase (MPO), with a perinuclear pattern. ANCA-associated diseases include Wegener's granulomatosis (WG), microscopic polyangiitis (MPA), Churg-Strauss syndrome (CSS) and a renal limited disease form that presents solely with necrotizing crescentic GN. Because of the strong ANCA association with the aforementioned diseases, ANCA has become a valuable diagnostic tool for clinicians. Moreover, a variety of in vitro and in vivo findings have established the causal role of ANCA in the disease development. Recently, the membrane-PR3 expression pattern on neutrophils was shown to have clinical significance, suggesting membrane-PR3 as a novel biomarker. Furthermore, the data demonstrate that membrane-PR3 expression is restricted to a stable subset of neutrophils. This subset is determined by the existence of the glycosylphosphatidylinositol (GPI)-anchored NB1 receptor (CD177). Better understanding of the PR3-NB1 interaction may have therapeutic implications with the development of more selective drugs.
Keywords:ANCA, Anti-GBM, Autoimmunity, Glomerulonephritis, Lupus Erythematosus, NB1, CD177
Source:Scandinavian Journal of Clinical and Laboratory Investigation
Publisher:Taylor & Francis
Number:Suppl. 241
Page Range:99-103
Official Publication:https://doi.org/10.1080/00365510802150232
PubMed:View item in PubMed

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