Behçet’s disease in children, an overview

Abstract

SummaryBD is a systemic inflammatory disease with a variable vasculitis. Paediatric onset is very rare and carries a strong genetic component. Oral ulcers and fever of unknown origin are frequent at onset and difficult to distinguish from other inflammatory disorders; therefore, expert opinion is still mandatory to recognize the disease early. An international expert consensus has recently proposed new classification criteria for children with BD. The clinical spectrum of BD is heterogeneous and influenced by gender, ethnicity and country of residence. Young males have the worst prognosis with significantly more frequent neurological, ocular and vascular involvement. BD treatment is aimed at alleviating inflammation. Among all drugs, TNFα inhibitors have become a standard to control severe ocular, neurological and digestive system involvement.

The Epidemiology of Behçet’s Disease

“…However, in few series, it was reported that 3.3 and 26% of the patients with BD were juvenile-onset BD [2]. The clinical symptoms are not diferent from adults but the diagnosis may be delayed in pediatric patients because of mild symptoms [2]. Juvenile onset patients had more familial cases compared with adult-onset patients [39].…”

“…The prevalence of juvenile BD is unknown. However, in few series, it was reported that 3.3 and 26% of the patients with BD were juvenile-onset BD [2]. The clinical symptoms are not diferent from adults but the diagnosis may be delayed in pediatric patients because of mild symptoms [2].…”

“…Behçet's disease (BD) is a chronic multisystem vasculitis, which afects any type and shape of blood vessels, particularly veins [1,2]. The disease was irst described in 1937 by a Turkish dermatologist, Hulusi Behçet, as a triple symptom complex, aphthous stomatitis, genital ulcers and relapsing uveitis [3].…”

Intestinal and neurological involvement in Behcet disease: a clinical case

“…BD is rare in paediatric age and the prevalence is unknown. It is difficult to make diagnosis under 16 years due to the heterogeneous clinical picture, such as oral and genital ulcers, erythema nodosum, superficial thrombophlebitis, acne and arthritis [6, 7]. Factors that contribute to the pathogenesis of BD include the host’s genetic profile and immune system, and environmental factors such as the gut microbiota.…”

Introductory Chapter: Behcet’s Disease - An Overview

“…The prevalence of juvenile BD is unknown. But in few series, it is reported between 3, 3 and 26% of BD patients were before age of 16 years [10]. BD affects the both gender equally [11].…”