without a clear outcome to date [1, 3] . It is a worldwide disease with a predilection for people living in the Far East, Middle East and the Mediterranean regions (former socalled Silk Route). ABD affects people of all ages, showing the highest prevalence of onset in the third decade of life. Both genders may be involved, though its clinical spectrum and severity display quite substantial differences between them [4, 5] . Patients with symptoms occurring up to the age of 16 are considered as

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... ses of juvenile-onset ABD (JABD) [6, 7] . JABD is relatively rare compared to its adult counterpart (AABD). Since the publication of the first article on the pediatric disease by Mundy and Miller in 1978 [8], several case reports and case studies have been described [6, 7, [9] [10] [11] [12] [13] [14] . However, reviews on this subject are rather scarce [6, [15] [16] [17] [18] . Epidemiology The epidemiology of JABD is difficult to estimate also because there is no formal agreement on either the age at the disease onset or the age at which the symptoms meet the older or current diagnostic and classification criteria [19, 20] .