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.

Keywords: Behçet’s syndrome; Childhood; Pediatric age; Personalized medicine; Uveitis.

Conflict of interest statement

The authors declare that this research was carried out in the absence of any personal, professional, or financial relationships that could potentially be construed as a conflict of interest.

© 2021. The Author(s).

Figures

Fig. 1

Distribution of age at onset for 64 patients with juvenile-onset Behçet’s syndrome (in accordance with the different age groups, expressed in years)

Fig. 2

Anatomical patterns of intraocular inflammation classified according to SUN criteria for 64 patients with juvenile-onset Behçet’s syndrome evaluated in our study

Fig. 3

Comparison of gender differences (excluding oral aphthae) for 64 patients with juvenile-onset Behçet’s syndrome evaluated in our study. EN erythema nodosum, CNS central nervous system, GI gastrointestinal involvement

Fig. 4

Therapeutic regimens administered in our cohort of 64 patients with juvenile-onset Behçet’s syndrome. cDMARDs conventional disease modifying anti-rheumatic drugs: CS corticosteroids

Fig. 5

Clinical differences between juvenile-onset Behçet’s syndrome and adult-onset Behçet’s syndrome expressed in percentages for each manifestation (excluding oral aphthae) developed during the disease course. EN erythema nodosum, CNS central nervous system, GI gastrointestinal involvement

Fig. 6

Differences in the development of major organ involvement between juvenile-onset and adult-onset Behçet’s syndrome throughout disease course