Juvenile Behçet's disease 2014-2024: multicenter study in Colombia

2014-2024年青少年白塞氏病:哥伦比亚多中心研究

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Abstract

INTRODUCTION: Juvenile Behçet's disease (JBD) is a rare multisystem inflammatory disorder that primarily affects the oral and genital mucosa, as well as vessels of any caliber. Between 4% and 26% of Behçet's disease cases begin before the age of 16. JBD remains underreported, with only 55 cases documented in Colombia in 2022. Its aetiology is uncertain, with possible infectious and genetic triggers. The wide range of clinical variability often leads to delayed diagnosis and treatment, increasing the risk of serious complications such as thrombosis and neurological damage. MATERIALS AND METHODS: A multicenter longitudinal study with retrospective data collection was conducted in patients under 18 years of age who met international diagnostic criteria for JBD between 2014 and 2024 in three Colombian cities: Cali, Bogotá, and Pereira. Clinical and paraclinical data were extracted from medical records and analysed via IBM SPSS Statistics v29 to obtain descriptive statistics and frequency distributions. RESULTS AND DISCUSSION: Fifteen patients were included, with a female predominance (60%). The mean age at symptom onset was 6.1 years, with a mean age at diagnosis of 11.8 years, revealing a diagnostic delay of 5.7 years. The most common manifestations were recurrent oral aphthosis (100%), genital ulcers (73.3%), arthralgia (60%), and neurological (40%) and vascular (20%) involvement-mainly venous thrombosis. The pathergy test was positive in 44.4% of patients, and viral antigens were detected in 46.7%. All patients received colchicine and corticosteroids; 86.7% required additional immunosuppressive therapy, and two patients were treated with biologics. CONCLUSION: Juvenile Behçet's disease in Colombia presents heterogeneous clinical presentations, with a high prevalence of mucocutaneous, musculoskeletal, and neurological involvement. A significant delay in diagnosis was observed, highlighting the need for increased clinical awareness and suspicion. Colchicine and corticosteroids are generally effective, although most patients require immunosuppressive agents, and some require biologic therapy. These findings emphasize the importance of timely diagnosis and individualized treatment strategies to reduce morbidity in affected children.

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