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Juvenile Behçet’s disease: a tertiary center experience

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Abstract

Objectives

Juvenile Behçet’s disease is a rare and severe disease of childhood characterized by a chronic inflammatory vasculitis. The aim of the present study is reporting demographic, clinical and therapeutic outcomes of juvenile Behçet’s disease in a tertiary center.

Methods

The retrospective study included patients who were diagnosed Behçet’s disease before 16 years. The demographic and clinical features, and diagnostic and therapeutic strategies of patients were recorded.

Results

Seventy-two patients with jBD were included in this study; 32 were male (44.4%). Thirty (41.7%) patients had BD cases in their family. We observed oral ulceration (100%), genital ulceration (68.1%), joint involvement (36.1%) and cutaneous manifestations (34.7%) as the most common clinical findings, respectively. As severe organ involvements, 20.8% ocular, 18.1% vascular and 15.3% neurologic pathologies were seen. All patients had colchicine. Corticosteroid (20.8%) was used to treat severe cases and acute attacks. Azathioprine (23.6%) was the main immunosuppressive agent and cyclophosphamide (8.3%) was applied initially for life-threatening conditions with pulse methylprednisolone.

Conclusion

In this cohort, the prevalence of genital ulceration and family history was high, and we observed less ocular involvement, a few permanent neurological morbidities and no death.

Key Points

• In the present study, there were acceptable permanent neurological involvements as morbidity and no mortality.

• It is important noticing and managing jBD in early phase in order to prevent the devastating results.

• The awareness of jBD provides timely treatment of patients.

• The positivity of family history and HLA B51 should alert the clinician about the incomplete cases.

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References

  1. Jennette JC, Falk RJ, Bacon PA, Basu N, Cid MC, Ferrario F et al (2013) 2012 revised International Chapel Hill Consensus Conference Nomenclature of Vasculitides. Arthritis Rheum 65:1–11

    Article  CAS  Google Scholar 

  2. Ozen S (2010) Pediatric onset Behçet disease. Curr Opin Rheumatol 22:585–589

    Article  Google Scholar 

  3. Yildiz M, Koker O, Adrovic A, Sahin S, Barut K, Kasapcopur O (2019) Pediatric Behçet’s disease - clinical aspects and current concepts. Eur J Rheumatol 7:1–10

    CAS  Google Scholar 

  4. Kone-Paut I (2016) Behcet’s disease in children, an overview. Pediatr Rheumatol. 14:10. https://doi.org/10.1186/s12969-016-0070-z

    Article  Google Scholar 

  5. Yurdakul S, Hamuryudan V, Yazici H (2004) Behçet syndrome. Curr Opin Rheumatol 16:38–42. https://doi.org/10.1097/00002281-200401000-00008

    Article  PubMed  Google Scholar 

  6. Gallizzi R, Pidone C, Cantarini L, Finetti M, Cattal-ini M, Filocamo G et al (2017) A national cohort study on pediatric Behcet’s disease: cross-sectional data from an Italian registry. Pediatr Rheumatol Online J 15:84

    Article  Google Scholar 

  7. International Study Group for Behcet’s Disease (1990) Criteria for diagnosis of Behcet’s disease. Lancet 335:1078–1080

  8. International Team for the Revision of the International Criteria for Behçet’s Disease (ITR-ICBD) (2014) The International Criteria for Behcet’s Disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol 28:338–347

  9. Kone-Paut I, Shahram F, Darce-Bello M, Can-tarini L, Cimaz R, Gattorno M et al (2016) Consensus classification criteria for paediatric Behcet’s disease from a prospective observational cohort: PEDBD. Ann Rheum Dis 75:958–964

    Article  Google Scholar 

  10. Lawton G, Bhakta BB, Chamberlain MA, Tennant A (2004) The Behcet’s disease activity index. Rheumatology 43:73–78

    Article  CAS  Google Scholar 

  11. Gezgin Yildirim D, Bakkaloğlu SA, Hasanreisoglu M, Buyan N (2020) Disease activity and outcomes in juvenile Behçet’s disease: 10 years’ experience of a single centre. Clin Exp Rheumatol 38:105–111

    PubMed  Google Scholar 

  12. Butbul Aviel Y, Batu ED, Sözeri B, Aktay Ayaz N, Baba L, Amarilyo G et al (2020) Characteristics of pediatric Behçet’s disease in Turkey and Israel: a cross-sectional cohort comparison. Semin Arthritis Rheum 50:515–520. https://doi.org/10.1016/j.semarthrit.2020.01.013

    Article  PubMed  CAS  Google Scholar 

  13. Atmaca L, Boyvat A, Yalcindag FN, Atmaca-Sonmez P, Gurler A (2011) Behçet disease in children. Ocul Immunol Inflamm 19:103–7

    Article  Google Scholar 

  14. Sungur GK, Hazirolan D, Yalvac I, Ozer PA, Yuksel D, Vural ET et al (2009) Clinical and demographic evaluation of Behçet disease among different paediatric age groups. Br J Ophthalmol 93:83–87

    Article  CAS  Google Scholar 

  15. Karincaoglu Y, Borlu M, Toker SC, Akman A, Onder M, Gunasti S et al (2008) Demographic and clinical properties of juvenile-onset Behçet’s disease: a controlled multicenter study. J Am Acad Dermatol 58:579–584

    Article  Google Scholar 

  16. Pain CE, Beresford MW, Fortune F, Lai ETC, Murphy R, Taylor-Robinson D, Brogan PA, Moots RJ (2021) Behçet’s syndrome in children and young people in the United Kingdom and the Republic of Ireland: a prospective epidemiological study. Rheumatology: keab084. https://doi.org/10.1093/rheumatology/keab084

  17. Shahram F, Nadji A, Akhlaghi M, Faezi ST, Chams-Davatchi C, Shams H et al (2018) Paediatric Behçet’s disease in Iran: report of 204 cases. Clin Exp Rheumatol 36:135–140

    PubMed  Google Scholar 

  18. Hamzaoui A, Jaziri F, Ben Salem T, Said Imed Ben Ghorbel F, Lamloum M, SmitiKhanfir M et al (2014) Comparison of clinical features of Behçet disease according to age in a Tunisian cohort. Acta Med Iran 52:748–51

    PubMed  Google Scholar 

  19. Nanthapisal S, Klein NI, Ambrose N, Eleftheriou D, Brogan PA (2016) Paediatric Behçet’s disease: a UK tertiary centre experience. Clin Rheumatol 35:2509–2516

    Article  Google Scholar 

  20. Makmur EL, Myers SH, Hanns L, Haskard DO, Brogan P, Ambrose N (2019) Comparing the clinical profile of adults and children with Behçet’s syndrome in the UK. Clin Exp Rheumatol 37 Suppl 121(6):48–51

    PubMed  Google Scholar 

  21. (2005) Familial Mediterranean fever (FMF) in Turkey: results of a nationwide multicenter study. Medicine 84:1–11. https://doi.org/10.1097/01.md.0000152370.84628.0c

  22. Stoimenis D, Petridis N, Papaioannou N (2013) Behçet’s disease, associated large vessel thrombosis, and coexistent thrombophilia: a distinct nosological entity? Case Rep Med: 740837. https://doi.org/10.1155/2013/740837

  23. Leiba M, Seligsohn U, Sidi Y, Harats D, Sela BA, Griffin JH, Livneh A, Rosenberg N, Gelernter I, Gur H, Ehrenfeld M (2004) Thrombophilic factors are not the leading cause of thrombosis in Behçet’s disease. Ann Rheum Dis 63:1445–1449. https://doi.org/10.1136/ard.2003.014241

    Article  PubMed  PubMed Central  CAS  Google Scholar 

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Authors and Affiliations

  1. Department of Pediatric of Rheumatology, Ankara City Hospital, Ankara, Turkey

    Zahide Ekici Tekin, Elif Çelikel, Fatma Aydin, Tuba Kurt, Müge Sezer, Nilüfer Tekgöz, Cüneyt Karagöl, Serkan Coşkun, Melike Mehveş Kaplan & Banu Çelikel Acar

  2. Ankara Şehir Hastanesi Çocuk Hastanesi, Üniversiteler Mahallesi 1604, Cadde No. 9, Çankaya/Ankara, Turkey

    Zahide Ekici Tekin

Authors
  1. Zahide Ekici Tekin
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  2. Elif Çelikel
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  3. Fatma Aydin
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  4. Tuba Kurt
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  5. Müge Sezer
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  6. Nilüfer Tekgöz
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  7. Cüneyt Karagöl
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  8. Serkan Coşkun
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  9. Melike Mehveş Kaplan
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  10. Banu Çelikel Acar
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Contributions

The study was conceived and designed, and patients’ data were collected and analysed, by ZET, TK, CK, SC and MMK. BCA, EC and FA supervised this work. The manuscript was written, and statistical analyses were performed, by ZET, TK, MS and PNT. All authors reviewed and accepted the final version of the manuscript.

Corresponding author

Correspondence to Zahide Ekici Tekin.

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Ethics approval

The Clinical Research Ethics Committee of Ankara City Hospital approved the study. All procedures were conducted according to the principles of the Declaration of Helsinki.

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Ekici Tekin, Z., Çelikel, E., Aydin, F. et al. Juvenile Behçet’s disease: a tertiary center experience. Clin Rheumatol 41, 187–194 (2022). https://doi.org/10.1007/s10067-021-05896-0

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  • DOI: https://doi.org/10.1007/s10067-021-05896-0

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