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.
Highlights
Behçet’s syndrome (BS) is a systemic multifactorial autoinflammatory disorder originally described with the triple symptom complex consisting of aphthous stomatitis, genitalTransplantation (DETO), University of Bari, Bari, Italy 5 Department of Experimental and Clinical Medicine, University of Florence, Florence, Italy 6 Clinical Pediatrics, Department of Molecular Medicine and Development, University of Siena, Siena, Italy 7 Ophthalmology Unit, Department of Medicine, Surgery and Neuroscience, University of Siena, Siena, Italy1 3 Vol.:(0123456789)Internal and Emergency Medicine (2021) 16:2163–2171 ulcers and uveitis [1,2,3,4]
BS is prevalent in the Eurasian populations, along the ancient trading “Silk Route”, while it is rarely encountered in Western populations [7]
Continuous variables were summarized with mean ± standard deviation (SD) or median ± interquartile range (IQR) as required, whereas categorical variables were reported as absolute frequencies
Summary
Given its systemic nature, any organ can be potentially affected, with vascular, gastrointestinal and central nervous system involvement being the most commonly reported [5]. With regard to specific clinical manifestations such as gastrointestinal involvement and pathergy reaction, different frequencies in different countries have been reported [8,9,10]. Male patients show a higher mortality, which is mainly due to central nervous system and large vessel involvement. Due to the relapsing–remitting nature of this syndrome, many years can pass between the first clinical manifestation and the full-blown disease. With the aim of shedding light on some blind spots that characterize pediatric BS we report our multicenter experience on a large sample of juvenile-onset BS, focusing on their clinical spectrum and assessing differences with adult patients as well as potential predictors of disease severity
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