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..
Medienart: |
E-Artikel |
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Erscheinungsjahr: |
2021 |
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Erschienen: |
2021 |
Enthalten in: |
Zur Gesamtaufnahme - volume:16 |
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Enthalten in: |
Internal and emergency medicine - 16(2021), 8 vom: 09. Apr., Seite 2163-2171 |
Sprache: |
Englisch |
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Beteiligte Personen: |
Sota, Jurgen [VerfasserIn] |
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Links: |
Volltext [kostenfrei] |
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BKL: | |
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Themen: |
Behçet’s syndrome |
Anmerkungen: |
© The Author(s) 2021 |
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doi: |
10.1007/s11739-021-02725-9 |
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funding: |
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Förderinstitution / Projekttitel: |
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PPN (Katalog-ID): |
OLC2128321131 |
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245 | 1 | 0 | |a Clinical profile and evolution of patients with juvenile-onset Behçet’s syndrome over a 25-year period: insights from the AIDA network |
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520 | |a 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. | ||
650 | 4 | |a Behçet’s syndrome | |
650 | 4 | |a Uveitis | |
650 | 4 | |a Childhood | |
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650 | 4 | |a Personalized medicine | |
700 | 1 | |a Rigante, Donato |0 (orcid)0000-0001-7032-7779 |4 aut | |
700 | 1 | |a Lopalco, Giuseppe |4 aut | |
700 | 1 | |a Emmi, Giacomo |4 aut | |
700 | 1 | |a Gentileschi, Stefano |4 aut | |
700 | 1 | |a Gaggiano, Carla |4 aut | |
700 | 1 | |a Ciarcia, Luisa |4 aut | |
700 | 1 | |a Berlengiero, Virginia |4 aut | |
700 | 1 | |a Mourabi, Mariam |4 aut | |
700 | 1 | |a Ricco, Nicola |4 aut | |
700 | 1 | |a Barneschi, Sara |4 aut | |
700 | 1 | |a Mattioli, Irene |4 aut | |
700 | 1 | |a Tosi, Gian Marco |4 aut | |
700 | 1 | |a Frediani, Bruno |4 aut | |
700 | 1 | |a Tarsia, Maria |4 aut | |
700 | 1 | |a di Scala, Gerardo |4 aut | |
700 | 1 | |a Vitale, Antonio |4 aut | |
700 | 1 | |a Iannone, Florenzo |4 aut | |
700 | 1 | |a Fabiani, Claudia |4 aut | |
700 | 1 | |a Cantarini, Luca |0 (orcid)0000-0002-7352-1275 |4 aut | |
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