ANCA (anti-neutrophil cytoplasm antibody)-associated small vessel vasculitis is an inflammatory condition associated with the production of autoantibodies to neutrophil cytoplasmic components. The disorder results in destruction of the microvasculature, infiltration of neutrophils into tissues, which is followed later by mononuclear cells, leading to injury and the formation of granulomatous lesions. Initiators for the disease are undetermined but a pro-inflammatory environment is required. Other influencing factors may include environmental triggers, genetic propensity or infectious agents. The primary cellular event in the condition involves the neutrophils, which are likely to be responsible for the majority of tissue injury. Binding of the autoantibody to neutrophils initiates cell activation via a complex intracellular signalling cascade, culminating in the release of pro-inflammatory mediators, proteolytic enzymes and reactive oxygen species. Adhesion of neutrophils to endothelial cells is observed in vitro and more investigations in this area may explain the focussing of the disease to certain vessels/tissues. Current treatment regimens have substantial toxicity. Although newer developments are an improvement there is still a pressing need for more targeted therapies, which could be provided by extrapolating information emerging from basic scientific research.
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February 2005
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Review Article|
January 21 2005
Translating basic science into patient therapy for ANCA-associated small vessel vasculitis
Julie M. WILLIAMS;
Julie M. WILLIAMS
1Renal Immunobiology, The Medical School, University of Birmingham, Edgbaston, Birmingham B15 2TT, U.K.
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Lavanya KAMESH;
Lavanya KAMESH
1Renal Immunobiology, The Medical School, University of Birmingham, Edgbaston, Birmingham B15 2TT, U.K.
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Caroline O. S. SAVAGE
1Renal Immunobiology, The Medical School, University of Birmingham, Edgbaston, Birmingham B15 2TT, U.K.
Correspondence: Professor Caroline O. S. Savage (email C.O.S.Savage@bham.ac.uk).
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Publisher: Portland Press Ltd
Received:
August 06 2004
Revision Received:
September 16 2004
Accepted:
October 25 2004
Accepted Manuscript online:
October 25 2004
Online ISSN: 1470-8736
Print ISSN: 0143-5221
The Biochemical Society
2005
Clin Sci (Lond) (2005) 108 (2): 101–112.
Article history
Received:
August 06 2004
Revision Received:
September 16 2004
Accepted:
October 25 2004
Accepted Manuscript online:
October 25 2004
Citation
Julie M. WILLIAMS, Lavanya KAMESH, Caroline O. S. SAVAGE; Translating basic science into patient therapy for ANCA-associated small vessel vasculitis. Clin Sci (Lond) 1 February 2005; 108 (2): 101–112. doi: https://doi.org/10.1042/CS20040232
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