Clusters in paediatric Behçet’s disease: a multicentre international study
Objectives Clinical features of Behçet’s disease (BD) exhibit significant variability, not only from patient to patient but also according to gender and geographical region. This study aims to describe the clinical characteristics, identify distinct clusters in a large cohort of paediatric BD patien...
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| Language: | English |
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BMJ Publishing Group
2025-07-01
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| Series: | RMD Open |
| Online Access: | https://rmdopen.bmj.com/content/11/3/e005335.full |
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| author | Seza Ozen Marco Gattorno Isabelle Kone-Paut Erbil Unsal Ezgi Deniz Batu Ummusen Kaya Akca Farhad Shahram Erdem Karabulut Betul Sozeri Seyedeh Tahereh Faezi Mustafa Çakan Massoomeh Akhlaghi Ozlem Akgun Esra Esen Caterina Matucci-Cerinic Ruya Torun Nuray Aktay Ayaz Ayşenur Paç Kısaarslan |
| author_facet | Seza Ozen Marco Gattorno Isabelle Kone-Paut Erbil Unsal Ezgi Deniz Batu Ummusen Kaya Akca Farhad Shahram Erdem Karabulut Betul Sozeri Seyedeh Tahereh Faezi Mustafa Çakan Massoomeh Akhlaghi Ozlem Akgun Esra Esen Caterina Matucci-Cerinic Ruya Torun Nuray Aktay Ayaz Ayşenur Paç Kısaarslan |
| author_sort | Seza Ozen |
| collection | DOAJ |
| description | Objectives Clinical features of Behçet’s disease (BD) exhibit significant variability, not only from patient to patient but also according to gender and geographical region. This study aims to describe the clinical characteristics, identify distinct clusters in a large cohort of paediatric BD patients and compare the clinical manifestations of patients across three geographical regions: Turkey, Europe and Iran.Method Patients with paediatric-onset BD (<18 years of age) from Turkey, Iran and European countries (France and Italy) were retrospectively evaluated. A follow-up period of at least 6 months was required for inclusion.Results The study included 600 patients (297 females, 49.5%), with cases from Turkey (n=231), Iran (n=306), France (n=44) and Italy (n=19). The most common presentations were mucocutaneous involvement (97.5%), followed by ocular (48.0%), musculoskeletal (43.2%), neurological (11.8%), vascular (11.5%), gastrointestinal (9.0%) and cardiac (2.0%) involvement. Ocular involvement was more prevalent in Iran, gastrointestinal involvement in Europe, and musculoskeletal and vascular involvement in Turkey compared with the other geographical regions. Seven distinct clusters of paediatric BD as vascular (cluster 1 (C1)), mucocutaneous only (C2), ocular (C3), gastrointestinal (C4), mixed (C5), neurologic and ocular (C6), and mucocutaneous-musculoskeletal cluster (C7) were identified, although there was some overlap in system involvements.Conclusions Our study supports the notion that BD may tend to present in certain clusters in children as well. Since BD is a complex disease with a multifactorial aetiology, involving the interaction of pathogenic pathways, classification of clusters presents a significant challenge. We have also shown that certain clinical features vary among geographical regions. |
| format | Article |
| id | doaj-art-5ea5ed5707f641a696d5059cda22eba7 |
| institution | DOAJ |
| issn | 2056-5933 |
| language | English |
| publishDate | 2025-07-01 |
| publisher | BMJ Publishing Group |
| record_format | Article |
| series | RMD Open |
| spelling | doaj-art-5ea5ed5707f641a696d5059cda22eba72025-08-20T02:42:35ZengBMJ Publishing GroupRMD Open2056-59332025-07-0111310.1136/rmdopen-2024-005335Clusters in paediatric Behçet’s disease: a multicentre international studySeza Ozen0Marco Gattorno1Isabelle Kone-Paut2Erbil Unsal3Ezgi Deniz Batu4Ummusen Kaya Akca5Farhad Shahram6Erdem Karabulut7Betul Sozeri8Seyedeh Tahereh Faezi9Mustafa Çakan10Massoomeh Akhlaghi11Ozlem Akgun12Esra Esen13Caterina Matucci-Cerinic14Ruya Torun15Nuray Aktay Ayaz16Ayşenur Paç Kısaarslan17Department of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, TurkeyUOC Rheumatology and Autoinflammatory Diseases, IRCCS Istituto Giannina Gaslini, Genova, ItalyDepartment of Paediatric Rheumatology and National French Reference Centre for Auto-Inflammatory Diseases and Inflammatory Amyloidosis (CEREMAIA), Bicêtre Hospital, Assistance publique-Hôpitaux de Paris, University Paris Saclay, Paris, FranceDepartment of Pediatric Rheumatology, Dokuz Eylül University, Faculty of Medicine, Izmir, TurkeyDepartment of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, TurkeyDepartment of Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, TurkeyRheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, IranDepartment of Biostatistics, Hacettepe University, Faculty of Medicine, Ankara, TurkeyDepartment of Pediatric Rheumatology, Umraniye Training and Research Hospital, Istanbul, TurkeyRheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, IranDepartment of Pediatric Rheumatology, Umraniye Training and Research Hospital, Istanbul, TurkeyRheumatology Research Center, Shariati Hospital, Tehran University of Medical Sciences, Tehran, IranDepartment of Pediatric Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, TurkeyDepartment of Pediatric Rheumatology, Erciyes University Faculty of Medicine, Kayseri, TurkeyUOC Rheumatology and Autoinflammatory Diseases, IRCCS Istituto Giannina Gaslini, Genova, ItalyDepartment of Pediatric Rheumatology, Dokuz Eylül University, Faculty of Medicine, Izmir, TurkeyDepartment of Pediatric Rheumatology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, TurkeyDepartment of Pediatric Rheumatology, Erciyes University Faculty of Medicine, Kayseri, TurkeyObjectives Clinical features of Behçet’s disease (BD) exhibit significant variability, not only from patient to patient but also according to gender and geographical region. This study aims to describe the clinical characteristics, identify distinct clusters in a large cohort of paediatric BD patients and compare the clinical manifestations of patients across three geographical regions: Turkey, Europe and Iran.Method Patients with paediatric-onset BD (<18 years of age) from Turkey, Iran and European countries (France and Italy) were retrospectively evaluated. A follow-up period of at least 6 months was required for inclusion.Results The study included 600 patients (297 females, 49.5%), with cases from Turkey (n=231), Iran (n=306), France (n=44) and Italy (n=19). The most common presentations were mucocutaneous involvement (97.5%), followed by ocular (48.0%), musculoskeletal (43.2%), neurological (11.8%), vascular (11.5%), gastrointestinal (9.0%) and cardiac (2.0%) involvement. Ocular involvement was more prevalent in Iran, gastrointestinal involvement in Europe, and musculoskeletal and vascular involvement in Turkey compared with the other geographical regions. Seven distinct clusters of paediatric BD as vascular (cluster 1 (C1)), mucocutaneous only (C2), ocular (C3), gastrointestinal (C4), mixed (C5), neurologic and ocular (C6), and mucocutaneous-musculoskeletal cluster (C7) were identified, although there was some overlap in system involvements.Conclusions Our study supports the notion that BD may tend to present in certain clusters in children as well. Since BD is a complex disease with a multifactorial aetiology, involving the interaction of pathogenic pathways, classification of clusters presents a significant challenge. We have also shown that certain clinical features vary among geographical regions.https://rmdopen.bmj.com/content/11/3/e005335.full |
| spellingShingle | Seza Ozen Marco Gattorno Isabelle Kone-Paut Erbil Unsal Ezgi Deniz Batu Ummusen Kaya Akca Farhad Shahram Erdem Karabulut Betul Sozeri Seyedeh Tahereh Faezi Mustafa Çakan Massoomeh Akhlaghi Ozlem Akgun Esra Esen Caterina Matucci-Cerinic Ruya Torun Nuray Aktay Ayaz Ayşenur Paç Kısaarslan Clusters in paediatric Behçet’s disease: a multicentre international study RMD Open |
| title | Clusters in paediatric Behçet’s disease: a multicentre international study |
| title_full | Clusters in paediatric Behçet’s disease: a multicentre international study |
| title_fullStr | Clusters in paediatric Behçet’s disease: a multicentre international study |
| title_full_unstemmed | Clusters in paediatric Behçet’s disease: a multicentre international study |
| title_short | Clusters in paediatric Behçet’s disease: a multicentre international study |
| title_sort | clusters in paediatric behcet s disease a multicentre international study |
| url | https://rmdopen.bmj.com/content/11/3/e005335.full |
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