Volume 12 Supplement 1

Proceedings of the 21st European Pediatric Rheumatology (PReS) Congress

Open Access

Vasculitis

  • Pavla Dolezalova1
Pediatric Rheumatology201412(Suppl 1):I20

https://doi.org/10.1186/1546-0096-12-S1-I20

Published: 17 September 2014

Primary systemic vasculitides (PSV) in children belong to the most challenging conditions in paediatric rheumatology practice. The spectrum of vasculitis affecting children as well as their clinical presentations differ from that of adults. Apart from relatively common Henoch-Shönlein purpura and Kawasaki disease, chronic PSV are extremely rare in childhood. They include mainly childhood polyarteritis nodosa (cPAN), Takayasu arteritis (cTA) and granulomatosis with polyangiitis (cGPA, formerly Wegener´s granulomatosis). From organ-specific diseases, primary CNS angiitis (PACNS) has been increasingly recognised in children over the recent years. Differential diagnosis of vasculitis covers wide spectrum of systemic diseases of inflammatory and neoplastic origin as well as etiologically heterogeneous group of non-inflammatory conditions reffered to as “pseudovasculitis”. In general, diagnostic confirmation of vasculitis requires histopathological or angiographic evidence of vascular involvement. Main diagnostic and treatment principles and classification criteria for the main vasculitides will be reviewed.

Principles of vasculitis assessment have been formulated by the OMERACT (Outcome Measures in Rheumatology) Vasculitis Working Group.1 Reversible features of acute morbidity directly related to the underlying inflammation are captured by the disease activity domain while irreversible consequences of previous active disease or long-term sequleae of treatment adverse effects form the basis of the disease damage domain. Physical function in terms of the degree of disability as well as psychosocial functioning including educational and vocational aspects are additional important components of patient-reported outcomes covered by the domain of health-related quality of life (HRQL). Paediatric-specific tools for vasculitis disease activity and damage assessment have been derived from adult instruments. Principles of Paediatric Vasculitis Activity Score (PVAS) and Paediatric Vasculitis Damage Index (PVDI) will be explained2,3.

Availability of childhood vasculitis classification and disease assessment tools has enabled initiation of the first paediatric vasculitis clinical trials. Ongoing international activities in the field of vasculitis include prospective disease registries that would allow update of disease classification and development of diagnostic criteria as well as improvement and validation of disease assessment tools.

Disclosure of interest

P Dolezalova Grant / Research Support from: Novartis, Roche, Abbvie, Pfizer, Consultant for: Roche.

Authors’ Affiliations

(1)
Paediatric Rheumatology Unit, Department of Paediatrics and Adolescent Medicine, General University Hospital and 1st Faculty of Medicine, Charles University in Prague

Copyright

© Dolezalova; licensee BioMed Central Ltd. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

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