Clinical profile and evolution of patients with juvenile-onset Behçet’s syndrome over a 25-year period: insights from the AIDA network

Abstract

Behçet’s syndrome (BS) represents an understudied topic in pediatrics: the main aims of our study were to characterize demographic and clinical features of a cohort of BS patients with juvenile-onset managed in three tertiary referral centers in Italy, evaluate their evolution in the long-term, and detect any potential differences with BS patients having an adult-onset. Medical records of 64 juvenile-onset and 332 adult-onset BS followed-up over a 2-year period were retrospectively analyzed and compared. Mean age ± SD of first symptom-appearance was 10.92 ± 4.34 years with a female-to-male ratio of 1.06:1. Mucocutaneous signs were the most frequent initial manifestations, followed by uveitis. Throughout the disease course, genital aphthae (76.56%) and pseudofolliculitis (40.63%) prevailed among the mucocutaneous signs, while major organ involvement was represented by gastrointestinal and ocular involvement (43.75 and 34.38%, respectively). No significant differences emerged for both mucocutaneous signs and specific major organ involvement between juvenile-onset and adult BS patients. After excluding nonspecific abdominal pain, juvenile-onset BS patients were less frequently characterized by the development of major organ involvement (p = 0.027). Logistic regression detected the juvenile-onset as a variable associated with reduced risk of long-term major organ involvement (OR 0.495 [0.263–0.932], p = 0.029). In our cohort, juvenile-onset BS resembled the clinical spectrum of adult-onset patients. Pediatric patients with a full-blown disease at onset showed a more frequent mucocutaneous involvement. In addition, patients with juvenile-onset seemed to develop less frequently major organ involvement and had an overall less severe disease course.

Behçet uveitis: Current practice and future perspectives

“…On the other hand 410 patients with 3 different disease entities distinct from BD were provided from the Eurofever Database as negative controls to test for the validity of the identified diagnostic criteria (12, 14, 46). Similar to adults, the most common presenting sign and often the first at a mean age of 8 to 9 years is recurrent, widespread multiple or single oral ulcers (44, 47, 48), with Sota et al deriving similar data from the AIDA Registry network (49). Genital ulcers are comparatively less frequent than in adults, however they are the second most common presenting sign in children and are seen predominantly in females.…”

“…Similar to adults, the most common presenting sign and often the first at a mean age of 8 to 9 years is recurrent, widespread multiple or single oral ulcers ( 44 , 47 , 48 ), with Sota et al deriving similar data from the AIDA Registry network ( 49 ). Genital ulcers are comparatively less frequent than in adults, however they are the second most common presenting sign in children and are seen predominantly in females.…”

Epidemiology and Risk Factors in Non-infectious Uveitis: A Systematic Review

“…The prevalence of Behçet disease has significant geographic variation with pooled global prevalence of 10.3 per 100,000 inhabitants, but with prevalence as high as 119 per 100,000 in Turkey to 2.1 per 100,000 for Northern Europe (31). Behçet disease can be associated with severe uveitis, with panuveitis being the most commonly observed location for uveitis (42). Occlusive retinal vasculitis is another common manifestation.…”

“…Occlusive retinal vasculitis is another common manifestation. A recent study by Sota et al compared 64 juvenile-onset and 332 adult-onset Behçet disease patients, and showed that those with juvenileonset Behçet disease (first manifestation of disease before age 16) have a lower prevalence of uveitis than adult-onset Behcet disease (42). Male patients may have higher risk for severe ocular involvement than female patients (43).…”

Treatment Options in Pediatric Behçet’s Disease

“…Behçet’s disease (BD) is a chronic systemic inflammatory disorder classically characterized by recurrent aphthous stomatitis, genital ulcers, and uveitis [ 1 ]. Nevertheless, this is a multi-system vasculitis able to involve blood vessels of all types and sizes, and any tissue or organ can be affected [ 2 ]. Worldwide frequency varies by geographical area, with the highest along the ‘Silk Route’ from the East of Asia to the Middle East including the countries around the Mediterranean basin.…”