British Paediatric Surveillance Unit Study
The study of children and young people with Behçet’s disease in the UK that is being funded by the BSS and Alder Hey Children’s Charity is due to start in April 2015. Anonymous information on all possible cases in hospitals in the UK and Ireland over a 13-month period will be sent to the study team for analysis, with a follow-up questionnaire a year later.
A leaflet with more information is available from the following link:
June 2017 – study update
British Paediatric Surveillance Unit (BPSU) study on Behçet’s syndrome in children and young people in the UK
The initial phase of the study of Behçet’s syndrome in children under the age of 16 in the UK (the first of its kind), covered the 25 month period from May 2015 to May 2017. During that time 149 notifications of suspected cases of Behçet’s syndrome were made by UK paediatricians and paediatric dermatologists.
At the time of writing this update, some initial questionnaires have yet to be received back by the study team, so this analysis is based on the completed questionnaires which have been received and processed to the end of May 2017 excluding any duplications and errors; a figure of 72. The data is anonymous as personal information is separated from clinical information after checking for duplication.
31 of the 72 cases are newly diagnosed cases of Behçet’s and the other 41 are from children who were diagnosed with Behçet’s previously. This information will be used to calculate the incidence (new cases) and prevalence (previous cases) of Behçet’s syndrome.
35 of the 72 cases are girls and the other 37 are boys. 60 of the children are from white backgrounds. The second largest ethnic background is Pakistani with a total of five children.
The average age at first symptom was 5 years and 6 months. The average age at diagnosis is 10 years and 2 months. The average delay between first symptom and formal diagnosis is 4 years and 2 months, so there is a significant delay between onset of first symptom and formal diagnosis of Behçet’s syndrome. However, this average covers a wide range of experiences, with there being a delay of less than one year in six cases and at the other end of the spectrum, there being a delay of 11 years in one case. Some of the delays can be explained by the fact that children may start with oral ulcers but do not develop other features of the disease until later.
Nearly all of the children, 71, had recurrent mouth ulcers and 49 had genital ulcers. Skin rashes were the third most common symptom, being reported in 35 cases. Eye, brain and blood vessel problems were less common, being reported in 18, 10 and three cases respectively. In 13 cases the child had a first degree relative with Behçet’s syndrome and in five cases the child had a more distant relative with Behçet’s syndrome.
Whilst eight children weren’t on any medication for their symptoms, 14 of the children were on topical treatment (mouthwash and/or steroid cream) only but another 47 were on systemic immunosuppressive treatment. 63 of the children were seen by three or more specialists and health professionals, depending on their symptoms.
When it comes to outcomes it was recorded that 14 children had their symptoms controlled by topical treatment (mouthwash and/or steroid cream) only and a further 29 had their symptoms controlled by immunosuppressive treatment. Whilst seven children were stable off medication, 18 children had active disease despite medication.
The follow up phase of this study will run until the end of May 2018 and focus on the outcome of Behçet’s syndrome in children one year after their case was notified to the initial study. The study team will provide further updates to the BSS as the study progresses.
For further information, please see the BPSU and BSS websites: http://www.rcpch.ac.uk/bpsu/bht and
Dr Clare Pain Consultant Paediatric Rheumatologist and study lead and Emma Latham, Research Assistant
Alder Hey Children’s NHS Foundation Trust, Liverpool, UK.