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Objectives

To define the outcome of paediatric patients with at least two symptoms of BD, and to obtain an appropriate definition of BD in patients< 16y.

Methods

An international expert committee has defined the criteria of inclusion. New patients or patients followed for a maximum of 3 years, who presented at least 2 symptoms of BD (among a list), and gave their informed consent were included, reviewed yearly.

Results

228 patients were included since 2008,(SR: 1), from 22 centres of 13 countries, median age of 12.5 y. Median age at first symptom was 7.2 y. Family history of BD was present in 22% and consanguinity in 4.5%. Median disease duration at inclusion was 4.7 y and from the first symptom to last visit was 7.5 y. Inclusion criteria plus oral aphtosis (mandatory) were (%): genital aphtosis 50, necrotic folliculitis 31, uveitis 28, familial history 22, pathergy positive 19, erythema nodosum 15, vascular 10 and retinal …