Abstract
Anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) are rare systemic autoimmune diseases characterised by inflammation of small blood vessels. Recent developments have been made in our understanding of the pathogenesis of these diseases, including the pathogenic role of ANCA, neutrophils and monocytes as mediators of injury, dysregulation of the complement system, and the role of T and B cells. Current treatment strategies for AAV are based on broad immunosuppression, which may have significant side effects. Advances in understanding of the pathogenesis of disease have led to the identification of new therapeutic targets which may lead to treatment protocols with less-toxic side effects. The aim of this review is to summarise current information and recent advances in understanding of the pathogenesis of AAV.
Highlights
The anti-neutrophil cytoplasm antibody (ANCA)-associated vasculitides (AAV) are a group of systemic autoimmune diseases characterised by inflammation of small blood vessels with multi-organ involvement, including the kidney, lung, nerves, gut, and ear, nose, and throat (ENT)
The two main target antigens of ANCA were identified as proteinase-3 (PR3) and myeloperoxidase (MPO), which are present in the granules of neutrophils and lysosomes of monocytes[3,4,5]
There are differing clinical syndromes associated with ANCA: granulomatosis with polyangiitis (GPA), microscopic polyangiitis (MPA), eosinophilic GPA (EGPA), and renal limited vasculitis
Summary
F1000 Faculty Reviews are written by members of the prestigious F1000 Faculty. They are commissioned and are peer reviewed before publication to ensure that the final, published version is comprehensive and accessible. Several studies have reported higher levels of BLyS in patients with AAV, and some have shown that levels correlated with disease activity and ANCA titre and decreased following treatment[76,77]. In a model of anti-MPO AAV in which mice are immunised with MPO followed by a subnephritogenic dose of anti-GBM globulin, depletion of CD4+ cells decreased disease severity with no effect on ANCA titres[86]. This model of disease has been used to identify pathogenic epitopes for both CD4+ and CD8+ T cells, and these epitopes have been used to induce disease[87,88]. In an in vivo model of xenografted nasal mucosa from patients with GPA to mice, tissue damage was shown to be mediated by fibroblasts[96]
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