A paper has now been published in Rheumatology . A link to the abstract can be found below. In addition a webinar has now been produced outlining some of the findings from the study which can be view below.
Lead investigator
Dr Clare Pain
Alder Hey Children's NHS Foundation Trust
Eaton Road
Liverpool L12 2AP
Email: behcet@alderhey.nhs.uk
About the study
Overview
Behçet’s syndrome is a rare multi-system inflammatory condition characterised by recurrent oral ulceration, genital ulceration, eye and skin involvement. There is currently very little data on incidence and prevalence of Behçet’s syndrome in children and young people, particularly within the UK and ROI population.
The purpose of this study is to establish the current UK and ROI incidence and prevalence of Behçet’s syndrome and to describe the burden of disease in children under 16 years of age in the UK.
Because of the perceived rareness of the condition, the investigators investigated both incidence and prevalence.
You can download the protocol card, including references, below.
Case definition
Children and young people up to but not including the age of 16 with possible Behçet’s syndrome (including new and follow-up cases) who have two or more of the following features not explained by an alternative diagnosis:
- oral aphthous ulceration
- skin involvement defined as erythema nodosum, pustulosis, folliculitis or acneiform lesions
- positive pathergy test (skin prick test)
- eye involvement (defined as uveitis and/or retinal vasculitis)
- genital ulceration
- family history of Behçet’s syndrome in a biological parent or sibling
- vascular involvement: arterial or venous thrombosis, thrombophlebitis and/or aneurysm
- neurological involvement suggestive of Behçet’s syndrome.
Exclusion criteria
Any child with above features due to an alternative confirmed diagnosis e.g. inflammatory bowel disease, coeliac disease, systemic lupus erythematosus, infection.
Reporting instructions
Clinicians were asked to report any patients seen in the last month with possible Behçet’s syndrome (including new and follow-up cases) meeting the case definition.
Webinar and publications
BPSU in collaboration with RCPCH held a free webinar focusing on Behcet’s syndrome on 10 May 2021. A panel of speakers presented the findings of this 2017 BPSU Behcet’s syndrome study. A recording of the event can be found below.
Published papers
C Pain, M Beresford, F Fortune, E Lai, R Murphy, D Taylor-Robinson, P Brogan, and R Moots. 'Behçet’s syndrome in children and young people in the United Kingdom & Republic of Ireland: a prospective epidemiological study'. Rheumatology. 2021
Duration
May 2015 to May 2017 (25 months of surveillance). Follow-up until May 2018 (12 month follow-up).
Funding
Behçet’s Syndrome Society (from funds raised by the Worshipful Company of Horner’s), Alder Hey Children’s charity and the study investigators.
Approval
This study was approved by NRES Committee North West - Liverpool East (REC reference: 15/NW/0035) and has been granted Section 251 HRA-CAG permission (CAG reference: 15/CAG/0103).