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Adalimumab in the treatment of pediatric Behçet’s disease: case-based review

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Abstract

Behçet’s disease (BD) is a systemic vasculitis affecting prominently the veins, which is usually diagnosed in adulthood, but can occur in children younger than 16 years in about 4–26% of cases. The therapy is based on several immune-suppressive drugs; in case of inadequate control and/or complications, the biologic therapy with anti-TNF drugs has been successfully used in adults. Here, we reported one pediatric case of BD with systemic (persistent/recurrent high fever), skin and mucosal manifestations (recurrent aphthous stomatitis, anal/penile ulcers, erythema nodosum and papulo-pustules), that were unresponsive to the conventional treatment with steroids and colchicine; however, he was successfully treated with adalimumab. Compared to adult patients, the experience with adalimumab in the treatment of pediatric BD is very limited. Indeed, through a systematic search in the medical literature, we retrieved 4 case reports and 2 case series, describing BD pediatric patients treated with adalimumab, in addition to three clinical studies including some BD children. The analysis and discussion of these available clinical experiences may indicate adalimumab as an effective and safe option to treat several forms of BD, in addition to BD-related chronic uveitis.

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References

  1. Seyahi E (2019) Phenotypes in Behçet’s syndrome. Intern Emerg Med. https://doi.org/10.1007/s11739-019-02046-y (Epub ahead of print)

    Article  PubMed  Google Scholar 

  2. Borlu M, Uksal U, Ferahbas A, Evereklioglu C (2006) Clinical features of Behcet’s disease in children. Int J Dermatol 45:713–716

    Article  PubMed  Google Scholar 

  3. Karincaoglu Y, Borlu M, Toker SC 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  PubMed  Google Scholar 

  4. Yazici H, Ugurlu S, Seyahi E (2012) Behçet’s disease, is it one condition? Clinic Rev Allerg Immunol 43:275–280

    Article  CAS  Google Scholar 

  5. Koné-Paut I (2016) Behçet’s disease in children, an overview. Pediatr Rheumatol Online J 14:10

    Article  PubMed  PubMed Central  Google Scholar 

  6. Hatemi G, Silman A, Bang D et al (2008) EULAR recommendations for the management of Behçet disease. Ann Rheum Dis 67:1656–1662

    Article  CAS  Google Scholar 

  7. Emmi G, Bettiol A, Silvestri E, Di Scala G, Becatti M, Fiorillo C, Prisco D (2018) Vascular Behçet’s syndrome: an update. Intern Emerg Med. https://doi.org/10.1007/s11739-018-1991-y (Epub ahead of print)

    Article  PubMed  Google Scholar 

  8. Gasparyan AY, Ayvazyan L, Blackmore H, Kitas GD (2011) Writing a narrative biomedical review: considerations for authors, peer reviewers, and editors. Rheumatol Int 31:1409–1417

    Article  PubMed  Google Scholar 

  9. Koné-Paut I, Shahram F, Darce-Bello M et al (2016) Consensus classification criteria for paediatric Behçet’s disease from a prospective observational cohort: pEDBD. Ann Rheum Dis 75:958–964

    Article  PubMed  Google Scholar 

  10. Caso F, Costa L, Rigante D, Lucherini OM et al (2014) Biological treatments in Behçet’s disease: beyond anti-TNF therapy. Mediators Inflamm 2014:107421

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Poddighe D, Cavagna L, Brazzelli V, Bruni P, Marseglia GL (2014) A hyper-ferritinemia syndrome evolving in recurrent macrophage activation syndrome, as an onset of amyopathic juvenile dermatomyositis: a challenging clinical case in light of the current diagnostic criteria. Autoimmun Rev 13:1142–1148

    Article  CAS  PubMed  Google Scholar 

  12. Vallet H, Seve P, Biard L et al (2016) Infliximab versus adalimumab in the treatment of refractory inflammatory uveitis: a multicenter study from the French uveitis network. Arthritis Rheumatol 68:1522–1530

    Article  CAS  PubMed  Google Scholar 

  13. Calvo-Río V, Blanco R, Beltrán E et al (2014) Anti-TNF-α therapy in patients with refractory uveitis due to Behçet’s disease: a 1-year follow-up study of 124 patients. Rheumatology (Oxford) 53:2223–2231

    Article  Google Scholar 

  14. Vitale A, Emmi G, Lopalco G et al (2017) Adalimumab effectiveness in Behçet’s disease: short and long-term data from a multicenter retrospective observational study. Clin Rheumatol 36:451–455

    Article  PubMed  Google Scholar 

  15. Tanida S, Inoue N, Kobayashi K et al (2015) Adalimumab for the treatment of Japanese patients with intestinal Behçet’s disease. Clin Gastroenterol Hepatol 13:940–948

    Article  CAS  PubMed  Google Scholar 

  16. Martín-Varillas JL, Calvo-Río V, Beltrán E et al (2018) Successful optimization of adalimumab therapy in refractory uveitis due to Behçet’s disease. Ophthalmology 125:1444–1451

    Article  PubMed  Google Scholar 

  17. Poddighe D, Romano M, Gattinara M, Gerloni V (2018) Biologics for the treatment of juvenile idiopathic arthritis. Curr Med Chem 25:5860–5893

    Article  CAS  PubMed  Google Scholar 

  18. Gallizzi R, Pidone C, Cantarini L et al (2017) A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry. Pediatr Rheumatol Online J 15:84

    Article  PubMed  PubMed Central  Google Scholar 

  19. Robinson AB, Gallentine WB, Rabinovich CE (2010) Pediatric neuro-Behçet’s disease responsive to adalimumab. Pediatr Neurol 43:291–293

    Article  PubMed  Google Scholar 

  20. Interlandi E, Leccese P, Olivieri I, Latanza L (2014) Adalimumab for treatment of severe Behçet’s uveitis: a retrospective long-term follow-up study. Clin Exp Rheumatol 32:S58–S62

    PubMed  Google Scholar 

  21. Pagnini I, Bondi T, Simonini G, Giani T, Marino A, Cimaz R (2015) Successful treatment with canakinumab of a paediatric patient with resistant Behçet’s disease. Rheumatology (Oxford) 54:1327–1328

    Article  Google Scholar 

  22. Marsili M, Marzetti V, Lucantoni M, Lapergola G, Gattorno M, Chiarelli F, Breda L (2016) Autoimmune sensorineural hearing loss as presenting manifestation of paediatric Behçet disease responding to adalimumab: a case report. Ital J Pediatr 42:81

    Article  PubMed  PubMed Central  Google Scholar 

  23. Lim KI, Yang DH, Ryoo E (2017) Behçet’s disease with multiple splenic abscesses in a child. Intest Res 15:422–428

    Article  PubMed  PubMed Central  Google Scholar 

  24. Atienza-Mateo B, Calvo-Río V, Beltrán E et al (2018) Anti-interleukin 6 receptor tocilizumab in refractory uveitis associated with Behçet’s disease: multicentre retrospective study. Rheumatology (Oxford) 57:856–864

    Article  CAS  Google Scholar 

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

    Article  PubMed  PubMed Central  Google Scholar 

  26. Simonini G, Taddio A, Cattalini M et al (2013) Superior efficacy of Adalimumab in treating childhood refractory chronic uveitis when used as first biologic modifier drug: adalimumab as starting anti-TNF-α therapy in childhood chronic uveitis. Pediatr Rheumatol Online J 11:16

    Article  PubMed  PubMed Central  Google Scholar 

  27. Deitch I, Amer R, Tomkins-Netzer O, Habot-Wilner Z, Friling R, Neumann R, Kramer M (2018) The effect of anti-tumor necrosis factor alpha agents on the outcome in pediatric uveitis of diverse etiologies. Graefes Arch Clin Exp Ophthalmol 256:801–808

    Article  CAS  PubMed  Google Scholar 

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Author notes

  1. Dimitri Poddighe and Zaure Mukusheva equally contributed to this article.

Authors and Affiliations

  1. Department of Medicine, School of Medicine, Nazarbayev University, Kerei-Zhanibek Str. 5/1, Astana, 010000, Kazakhstan

    Dimitri Poddighe & Kaisar Dauyey

  2. Department of Pediatric Rheumatology, National Research Center of Mother and Child Health, University Medical Center, Astana, Kazakhstan

    Zaure Mukusheva & Maikesh Assylbekova

Authors
  1. Dimitri Poddighe
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  2. Zaure Mukusheva
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  3. Kaisar Dauyey
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  4. Maikesh Assylbekova
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Contributions

DP conceived and wrote the article, and provided contribution to the clinical management; ZM and MA cooperated in the clinical management and provided important intellectual contributions; KD cooperated in the literature search and patient’s management.

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Correspondence to Dimitri Poddighe.

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Poddighe, D., Mukusheva, Z., Dauyey, K. et al. Adalimumab in the treatment of pediatric Behçet’s disease: case-based review. Rheumatol Int 39, 1107–1112 (2019). https://doi.org/10.1007/s00296-019-04300-0

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  • DOI: https://doi.org/10.1007/s00296-019-04300-0

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