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

Jurgen Sota, Donato Rigante, Giuseppe Lopalco, Giacomo Emmi, Stefano Gentileschi, Carla Gaggiano, Luisa Ciarcia, Virginia Berlengiero, Mariam Mourabi, Nicola Ricco, Sara Barneschi, Irene Mattioli, Gian Marco Tosi, Bruno Frediani, Maria Tarsia, Gerardo di Scala, Antonio Vitale, Florenzo Iannone, Claudia Fabiani, Luca Cantarini

Research output: Contribution to journalArticlepeer-review


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.

Original languageEnglish
Pages (from-to)2163-2171
Number of pages9
JournalInternal and Emergency Medicine
Issue number8
Publication statusPublished - Nov 2021


  • Behçet’s syndrome
  • Childhood
  • Pediatric age
  • Personalized medicine
  • Uveitis

ASJC Scopus subject areas

  • Internal Medicine
  • Emergency Medicine


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