Abstract
The discovery of autoantibodies to components of neutrophil cytoplasm, widely known as anti-neutrophil cytoplasmic antibodies (ANCA), has led to great advances in the diagnosis and monitoring of patients with primary systemic vasculitis. The antigenic targets of ANCA have been identified as neutrophil granule constituents, and it appears that the specificity of the antibodies is associated with particular clinical characteristics. The presence of ANCA is associated with active disease, and rising concentrations may herald a relapse. There is evidence from in vitro studies that ANCA can activate neutrophils and lead to endothelial cell damage, suggesting that they may have a pathogenic role in vasculitis. More recently, ANCA have been described in a limited range of other diseases. The specificity of these autoantibodies is less well defined, but includes targets other than those recognised in systemic vasculitis. Data on their prevalence and their relationship to disease activity is conflicting, and there is no experimental evidence for pathogenicity.
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Pusey, C.D., Gaskin, G. (1993). Disease Associations with Anti-Neutrophil Cytoplasmic Antibodies. In: Gross, W.L. (eds) ANCA-Associated Vasculitides. Advances in Experimental Medicine and Biology, vol 336. Springer, Boston, MA. https://doi.org/10.1007/978-1-4757-9182-2_25
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DOI: https://doi.org/10.1007/978-1-4757-9182-2_25
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