BPSU - Incidence and prevalence of Behçet’s syndrome in children and young people <16 years of age in the UK and ROI

Surveillance of the incidence and prevalence of Behçet’s syndrome in children and young people <16 years of age commenced in May 2015. The study team hope to ascertain the incidence and prevalence of Behçet’s syndrome in children and young people. They also hope to examine the clinical presentation and management of the syndrome.

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Lead investigator

Clare Pain.png

Dr Clare Pain
Alder Hey Children's NHS Foundation Trust
Eaton Road
Liverpool
L12 2AP
Tel: 0151 282 4521

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 plan to investigate both incidence AND prevalence.

Case definition: Please report any patients seen in the last month with possible Behçet’s syndrome (including new and follow-up cases) as defined as:

Children and young people up to but not including the age of 16 who have 2 of 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.

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.

Ethical approval

This study has been 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). 

Support group

Further information