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Vasculitis in children

Eleftheriou, D; Brogan, PA; (2018) Vasculitis in children. Paediatrics and Child Health , 28 (2) pp. 57-63. 10.1016/j.paed.2017.10.009. Green open access

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Abstract

Systemic vasculitis is characterized by blood vessel inflammation which may lead to tissue injury from vascular stenosis, occlusion, aneurysm, and/or rupture. Apart from relatively common vasculitides such as IgA Vasculitis (IgAV; previously referred to as Henoch-Schőnlein Purpura [HSP]) and Kawasaki disease (KD), most of the primary vasculitic syndromes are rare in childhood, but are associated with significant morbidity and mortality. The cause of the majority of vasculitides is unknown, although it is likely that a complex interaction between environmental factors such as infections and inherited host responses trigger the disease and determine the vasculitis phenotype. Several genetic polymorphisms in vasculitis have now been described. Treatment regimens continue to improve, with the use of different immunosuppressive medications and newer therapeutic approaches such as biologic agents. Randomized control studies involving predominantly adults have recently recruited children with vasculitis too; but rare disease trial design is required for paediatric specific trials. The SHARE (Single-Hub Access for Pediatric Rheumatology in Europe) project has recently provided guidance on management of rare paediatric rheumatic diseases including the vasculitides. This article provides an overview of paediatric vasculitides with emphasis on presenting features, current insights on aetiopathogenesis and treatment advances.

Type: Article
Title: Vasculitis in children
Open access status: An open access version is available from UCL Discovery
DOI: 10.1016/j.paed.2017.10.009
Publisher version: http://dx.doi.org/10.1016/j.paed.2017.10.009
Language: English
Additional information: © 2017 Elsevier Ltd. This version is the author accepted manuscript. For information on re-use, please refer to the publisher’s terms and conditions.
Keywords: child, Henoch-Schőnlein Purpura, IgA Vasculitis, Kawasaki disease, vasculitis
UCL classification: UCL
UCL > Provost and Vice Provost Offices
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 Population Health Sciences
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health
UCL > Provost and Vice Provost Offices > School of Life and Medical Sciences > Faculty of Population Health Sciences > UCL GOS Institute of Child Health > Infection, Immunity and Inflammation Dept
URI: https://discovery.ucl.ac.uk/id/eprint/10052045
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