Clusters in paediatric Behçet's disease: a multicentre international study.

Abstract

Objectives: Clinical features of Behçet's disease (BD) exhibit significant variability, not only from patient to patient but also according to gender and geographical region. This study aims to describe the clinical characteristics, identify distinct clusters in a large cohort of paediatric BD patients and compare the clinical manifestations of patients across three geographical regions: Turkey, Europe and Iran.

Method: Patients with paediatric-onset BD (<18 years of age) from Turkey, Iran and European countries (France and Italy) were retrospectively evaluated. A follow-up period of at least 6 months was required for inclusion.

Results: The study included 600 patients (297 females, 49.5%), with cases from Turkey (n=231), Iran (n=306), France (n=44) and Italy (n=19). The most common presentations were mucocutaneous involvement (97.5%), followed by ocular (48.0%), musculoskeletal (43.2%), neurological (11.8%), vascular (11.5%), gastrointestinal (9.0%) and cardiac (2.0%) involvement. Ocular involvement was more prevalent in Iran, gastrointestinal involvement in Europe, and musculoskeletal and vascular involvement in Turkey compared with the other geographical regions. Seven distinct clusters of paediatric BD as vascular (cluster 1 (C1)), mucocutaneous only (C2), ocular (C3), gastrointestinal (C4), mixed (C5), neurologic and ocular (C6), and mucocutaneous-musculoskeletal cluster (C7) were identified, although there was some overlap in system involvements.

Conclusions: Our study supports the notion that BD may tend to present in certain clusters in children as well. Since BD is a complex disease with a multifactorial aetiology, involving the interaction of pathogenic pathways, classification of clusters presents a significant challenge. We have also shown that certain clinical features vary among geographical regions.