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Genetics and pathogenesis of small-vessel vasculitis

Salama, AD; (2018) Genetics and pathogenesis of small-vessel vasculitis. Best Practice & Research Clinical Rheumatology , 32 (1) pp. 21-30. 10.1016/j.berh.2018.10.002. Green open access

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Abstract

Small-vessel vasculitides are uncommon autoimmune diseases characterised by inflammation and necrosis of arterioles, capillaries and venules, frequently described as various (previously eponymous) clinical syndromes. Some are associated with vessel wall immune complex deposition, whereas others are pauci-immune but paradoxically often associated with circulating anti-neutrophil cytoplasmic antibodies (ANCA). Most is known about the pathogenesis of the pauci-immune ANCA-associated syndromes, which are gradually becoming better understood with regard to their genetic predisposition and the critical pathways mediating disease initiation, as well as their particular phenotypic features. Through better understanding of key cellular and molecular players, we have been able to develop novel biomarkers and treatment strategies, which should translate to improved diagnostics, treatment protocols and, ultimately, better patient outcomes. These conditions are treatable but not yet curable, although it is clear that patients may follow different disease courses, which for some include restoration of their pre-morbid immune status.

Type: Article
Title: Genetics and pathogenesis of small-vessel vasculitis
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.berh.2018.10.002
Publisher version: http://doi.org/10.1016/j.berh.2018.10.002
Language: English
Additional information: This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: ANCA, Immunity, Vasculitis
UCL classification: UCL
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Medical Sciences > Div of Medicine > Renal Medicine
URI: https://discovery.ucl.ac.uk/id/eprint/10061785
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