Oral Ulceration in Behçets disease (BD) : International Delphi Study

Objectives: Oral Ulceration (OU) may present as a hallmark diagnosis or be part of sytemic conditions such as BD, Inflammatory Bowel Disease (IBD), Mucous Membrane Pemphigoid (MMP) and Aphtous Ulceration (AU) amongst others.
The aim of this study is
1 - To test the hypothesis that BD ulcers defer from other causes of oral ulceration including Recurrent Apthous Ulceration (RAS)
2- To define the clinical parameters that describe BD ulcers which will allow differentiation amongst other causes of oral ulceration including IBD, MMP and RAS
3- To develop clinical guidelines for BD experts on when the suspition of BD should be consider
Methods: Ethical approval from the University of Bimingham was granted. 18 BD experts were invited to take part of the study via email. 12 participants from 7 countries took part, consent form was signed. 5 medical specialties were involved, showing heterogenity in the cohort avoiding Oral Medicine only expert bias. 4 rounds of questionnaires were sent. In Round 1 and 2, the participants agreed in which clinical pictures could be consistent with a diagnosis of BD by clinical phenotype only. In Round 3 and 4, the clinical parameters were set. Agreement of 80% or above was reached in each round.
Results: Experts agreement in Round 1 and 2 showed a clear differentiation (98%) on BD ulcers from other causes of OU such as MMP or IBD but less agreement (73%) in differentiating amongst RAS ulcers. In Round 3 and 4, 10 clinical parameters necessary to differenciate BD ulcers from other causes of OU were defined
Experts did not find it possible to diferentiate RAS diagnosis from BD ulcers using clinical phenotype via clinical pictures only.(73%)
Conclusions: This is the first attempt to define the clinical parameters of BD ulcers by International Delphi Study which have now been set as well as explore the possibility that BD ulcers could defer from RAS ulcers. This novel and ongoing international collaboration aims to consequently develop clinical guidelines to non BD experts in hope to aid to earlier diagnosis and consequently increase patient.s quality of life.