Diagnostic/classification criteria in pediatric Behçet’s disease

Abstract

Behçet's disease (BD) is a variable vessel vasculitis characterized by recurrent oral and genital aphthosis accompanied by skin, ocular, gastrointestinal, neurologic, and articular involvement. BD is not common in childhood and the disease characteristics considerably differ between adults and children. 18 diagnostic/classification criteria have been published for BD to date. The pediatric BD (PEDBD) criteria, published in 2015, focused on pediatric BD, while the others mainly based on adult studies and are not validated for children. The aim of this review is to summarize the data about diagnostic/classification criteria for BD and to discuss the use and performance of the current criteria in pediatric BD. The covered topics are the characteristics of the diagnostic/classification criteria sets for BD, the factors restricting the universal use/acceptance of these criteria, and pediatric studies testing the performance of BD criteria sets. Having valid and universally accepted criteria with high performance is very important in pediatric BD as they help us determine patients for our studies and guide us through our clinical practice. There are less than 10 pediatric studies testing the performances of BD diagnostic/classification criteria. Their results suggest that revised ICBD (The International Criteria for BD) has the highest sensitivity, while ISG (The International Study Group) criteria remain as the most specific criteria set. Larger multinational pediatric BD cohorts with adequate control groups are required to compare the performance of the different criteria sets in children and to improve the performance of the existing PEDBD criteria.

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