• 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, and Luca Cantarini.
• Research Center of Systemic Auto Inflammatory Diseases, Behçet's Disease and Rheumatology-Ophthalmology Collaborative Uveitis Center, Rheumatology Unit, Policlinico "Le Scotte", Department of Medical Sciences, Surgery and Neurosciences, University of Siena, Viale Bracci 16, 53100, Siena, Italy.
• Intern Emerg Med. 2021 Nov 1; 16 (8): 2163-2171.

AbstractBehçet'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.© 2021. The Author(s).

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