Clinical profile and evolution of patients with juvenile-onset Behçet's syndrome over a 25-year period : insights from the AIDA network
© 2021. The Author(s)..
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. Nov., Seite 2163-2171 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Sota, Jurgen [VerfasserIn] |
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| Links: |
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| Themen: |
Behçet’s syndrome |
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| Anmerkungen: |
Date Completed 29.11.2021 Date Revised 18.02.2022 published: Print-Electronic Citation Status MEDLINE |
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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): |
NLM323905501 |
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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 |b insights from the AIDA network |
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| 520 | |a © 2021. The Author(s). | ||
| 520 | |a 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 Journal Article | |
| 650 | 4 | |a Behçet’s syndrome | |
| 650 | 4 | |a Childhood | |
| 650 | 4 | |a Pediatric age | |
| 650 | 4 | |a Personalized medicine | |
| 650 | 4 | |a Uveitis | |
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| 700 | 1 | |a Lopalco, Giuseppe |e verfasserin |4 aut | |
| 700 | 1 | |a Emmi, Giacomo |e verfasserin |4 aut | |
| 700 | 1 | |a Gentileschi, Stefano |e verfasserin |4 aut | |
| 700 | 1 | |a Gaggiano, Carla |e verfasserin |4 aut | |
| 700 | 1 | |a Ciarcia, Luisa |e verfasserin |4 aut | |
| 700 | 1 | |a Berlengiero, Virginia |e verfasserin |4 aut | |
| 700 | 1 | |a Mourabi, Mariam |e verfasserin |4 aut | |
| 700 | 1 | |a Ricco, Nicola |e verfasserin |4 aut | |
| 700 | 1 | |a Barneschi, Sara |e verfasserin |4 aut | |
| 700 | 1 | |a Mattioli, Irene |e verfasserin |4 aut | |
| 700 | 1 | |a Tosi, Gian Marco |e verfasserin |4 aut | |
| 700 | 1 | |a Frediani, Bruno |e verfasserin |4 aut | |
| 700 | 1 | |a Tarsia, Maria |e verfasserin |4 aut | |
| 700 | 1 | |a di Scala, Gerardo |e verfasserin |4 aut | |
| 700 | 1 | |a Vitale, Antonio |e verfasserin |4 aut | |
| 700 | 1 | |a Iannone, Florenzo |e verfasserin |4 aut | |
| 700 | 1 | |a Fabiani, Claudia |e verfasserin |4 aut | |
| 700 | 1 | |a Cantarini, Luca |e verfasserin |4 aut | |
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