Behçet syndrome in children and adults : discovering similarities and differences by a comparative study
© The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissionsoup.com..
OBJECTIVE: Behçet's syndrome (BS) is a rare disorder with a relapsing-remitting course. Clinical variance across geographical regions and different age groups has been observed. This study matched the demographic, clinical and treatment features of adult- and juvenile-onset BS in the Italian population.
METHODS: Two clinical databases of BS patients were compared. The paediatric BS database was collected at the Meyer Children's Hospital, Florence, while the adult BS database was collected at the Careggi University Hospital, Florence.
RESULTS: A familiar predisposition for BS was significantly more frequent in the paediatric cohort (3/33 vs 1/165, P = 0.015). No difference emerged in terms of prevalence of HLA-B51 positivity. The proportion of patients meeting the revised ICBD and/or the ISG criteria at BS diagnosis was comparable in the two cohorts. No significant difference emerged between the two cohorts in terms of muco-cutaneous, ocular and neurological involvement, and gastrointestinal symptoms. Articular manifestations resulted as more common in the paediatric cohort, whereas venous vascular events were more frequent in the adult cohort. Regarding treatment strategy, paediatric patients more frequently received no treatment or corticosteroid monotherapy. Conversely, the use of DMARDs, both traditional and biologic, was significantly higher in the adult cohort.
CONCLUSION: Remarkable differences between juvenile-onset and adult-onset BS, both in terms of gender, familiar predisposition and clinical manifestations have been observed and a different therapeutic approach in the real clinical practice of the two settings emerged. Prospective, comparison studies with a longer follow-up are encouraged to provide further data about the disease course for juvenile- and adult-onset BS.
| Medienart: |
E-Artikel |
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| Erscheinungsjahr: |
2023 |
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| Erschienen: |
2023 |
| Enthalten in: |
Zur Gesamtaufnahme - volume:62 |
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| Enthalten in: |
Rheumatology (Oxford, England) - 62(2023), SI2 vom: 23. Feb., Seite SI189-SI195 |
| Sprache: |
Englisch |
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| Beteiligte Personen: |
Mastrolia, Maria Vincenza [VerfasserIn] |
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| Links: |
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| Themen: |
Adrenal Cortex Hormones |
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| Anmerkungen: |
Date Completed 27.02.2023 Date Revised 27.03.2023 published: Print Citation Status MEDLINE |
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| doi: |
10.1093/rheumatology/keac347 |
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| funding: |
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| Förderinstitution / Projekttitel: |
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| 500 | |a Citation Status MEDLINE | ||
| 520 | |a © The Author(s) 2022. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: journals.permissionsoup.com. | ||
| 520 | |a OBJECTIVE: Behçet's syndrome (BS) is a rare disorder with a relapsing-remitting course. Clinical variance across geographical regions and different age groups has been observed. This study matched the demographic, clinical and treatment features of adult- and juvenile-onset BS in the Italian population | ||
| 520 | |a METHODS: Two clinical databases of BS patients were compared. The paediatric BS database was collected at the Meyer Children's Hospital, Florence, while the adult BS database was collected at the Careggi University Hospital, Florence | ||
| 520 | |a RESULTS: A familiar predisposition for BS was significantly more frequent in the paediatric cohort (3/33 vs 1/165, P = 0.015). No difference emerged in terms of prevalence of HLA-B51 positivity. The proportion of patients meeting the revised ICBD and/or the ISG criteria at BS diagnosis was comparable in the two cohorts. No significant difference emerged between the two cohorts in terms of muco-cutaneous, ocular and neurological involvement, and gastrointestinal symptoms. Articular manifestations resulted as more common in the paediatric cohort, whereas venous vascular events were more frequent in the adult cohort. Regarding treatment strategy, paediatric patients more frequently received no treatment or corticosteroid monotherapy. Conversely, the use of DMARDs, both traditional and biologic, was significantly higher in the adult cohort | ||
| 520 | |a CONCLUSION: Remarkable differences between juvenile-onset and adult-onset BS, both in terms of gender, familiar predisposition and clinical manifestations have been observed and a different therapeutic approach in the real clinical practice of the two settings emerged. Prospective, comparison studies with a longer follow-up are encouraged to provide further data about the disease course for juvenile- and adult-onset BS | ||
| 650 | 4 | |a Journal Article | |
| 650 | 4 | |a Behçet syndrome | |
| 650 | 4 | |a epidemiology | |
| 650 | 4 | |a immunosuppressants | |
| 650 | 4 | |a paediatric/juvenile rheumatology | |
| 650 | 4 | |a vasculitis | |
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| 650 | 7 | |a Adrenal Cortex Hormones |2 NLM | |
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| 700 | 1 | |a Marrani, Edoardo |e verfasserin |4 aut | |
| 700 | 1 | |a Maccora, Ilaria |e verfasserin |4 aut | |
| 700 | 1 | |a Taddei, Emilia |e verfasserin |4 aut | |
| 700 | 1 | |a Pagnini, Ilaria |e verfasserin |4 aut | |
| 700 | 1 | |a Canfora, Maria |e verfasserin |4 aut | |
| 700 | 1 | |a Emmi, Giacomo |e verfasserin |4 aut | |
| 700 | 1 | |a Silvestri, Elena |e verfasserin |4 aut | |
| 700 | 1 | |a Prisco, Domenico |e verfasserin |4 aut | |
| 700 | 1 | |a Simonini, Gabriele |e verfasserin |4 aut | |
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