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Development of de novo major involvement during follow-up in Behçet’s syndrome

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Abstract

The primary aim of the study was to evaluate the incidence of de novo major involvement during follow-up in a cohort of patients with Behçet’s syndrome (BS); the secondary aim was to analyse the epidemiological profile and the long-term outcome of those patients who developed new major involvement. Among our cohort of 120 BS patients, we evaluated all subjects who had no major organ involvement during the early years of their disease; specifically, at disease onset, the 52 % of the cohort presented a prevalent mucocutaneous involvement. The primary outcomes were represented by the following: Hatemi et al. (Rheum Dis Clin North Am 39(2):245–61, 2013) the incidence of de novo major involvement during the follow-up and Hatemi et al. (Clin Exp Rheumatol 32(4 Suppl 84):S112–22, 2014) the use of immunosuppressive drugs during the follow-up. We have defined the development of de novo major involvement during the follow-up as the occurrence of severe ocular, vascular or CNS involvement after a latency period from the diagnosis of at least 3 years. Among 62 patients characterized by a mild onset of disease, we observed that after at least 3 years from the diagnosis, 21 BS patients (34 %) still developed serious morbidities. Specifically, three patients developed ocular involvement, nine patients developed neurological involvement and nine patients presented vascular involvement. Comparing main epidemiological and clinical findings of the two groups, we observed that patients who developed de novo major involvement were more frequently males and younger; furthermore, 95 % of these patients were characterized by a young onset of disease (p < 0.001). Being free of major organ complication in the first years of BS is not necessary a sign of a favourable outcome. Globally, the development of de novo major involvement during the coursfce of BS suggests that a tight control is strongly recommended during the course of the disease.

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References

  1. Hatemi G, Yazici Y, Yazici H (2013) Behçet's syndrome. Rheum Dis Clin North Am 39(2):245–261

    Article  PubMed  Google Scholar 

  2. Hatemi G, Seyahi E, Fresko I, Talarico R, Hamuryudan V (2014) Behçet’s syndrome: a critical digest of the 2013–2014 literature. Clin Exp Rheumatol 32(4 Suppl 84):S112–S122

    PubMed  Google Scholar 

  3. Koné-Paut I, Darce-Bello M, Shahram F, Gattorno M, Cimaz R, Ozen S, Cantarini L, Tugal-Tutktun I, Assaad-Khalil S, Hofer M, Kuemmerle-Deschner J, Benamour S, Al Mayouf S, Pajot C, Anton J, Faye A, Bono W, Nielsen S, Letierce A, Tran TA, PED-BD International Expert Committee (2011) Registries in rheumatological and musculoskeletal conditions. Paediatric Behçet’s disease: an international cohort study of 110 patients. One-year follow-up data. Rheumatology (Oxford) 50:184–188. doi:10.1093/rheumatology/keq324

    Article  Google Scholar 

  4. Vitale A, Rigante D, Lopalco G, Brizi MG, Caso F, Franceschini R, Denaro R, Galeazzi M, Punzi L, Iannone F, Lapadula G, Simpatico A, Marrani E, Costa L, Cimaz R, Cantarini L (2014) Serum amyloid-A in Behçet’s disease. Clin Rheumatol 33(8):1165–1167

    Article  PubMed  Google Scholar 

  5. Hatemi G, Yazici H (2011) Behçet’s syndrome and micro-organisms. Best Pract Res Clin Rheumatol 25(3):389–406

    Article  PubMed  Google Scholar 

  6. Yazici H, Ugurlu S, Seyahi E (2012) Behçet syndrome: is it one condition? Clin Rev Allergy Immunol 43(3):275–280

    Article  PubMed  CAS  Google Scholar 

  7. Cantarini L, Vitale A, Lucherini OM, De Clemente C, Caso F, Costa L, Emmi G, Silvestri E, Magnotti F, Maggio MC, Prinzi E, Lopalco G, Frediani B, Cimaz R, Galeazzi M, Rigante D (2014) The labyrinth of autoinflammatory disorders: a snapshot on the activity of a third-level center in Italy. Clin Rheumatol

  8. Cantarini L, Vitale A, Borri M, Galeazzi M, Franceschini R (2012) Successful use of canakinumab in a patient with resistant Behçet’s disease. Clin Exp Rheumatol 30(3 Suppl 72):S115

    PubMed  CAS  Google Scholar 

  9. Cantarini L, Vitale A, Scalini P, Dinarello CA, Rigante D, Franceschini R, Simonini G, Borsari G, Caso F, Lucherini OM, Frediani B, Bertoldi I, Punzi L, Galeazzi M, Cimaz R (2013) Anakinra treatment in drug-resistant Behcet’s disease: a case series. Clin Rheumatol. doi:10.1007/s10067-013-2443-8

  10. Cantarini L, Lopalco G, Caso F, Costa L, Iannone F, Lapadula G et al (2015) Effectiveness and tuberculosis-related safety profile of interleukin-1 blocking agents in the management of Behçet's disease. Autoimmun Rev 14:1–9

    Article  PubMed  CAS  Google Scholar 

  11. International Study Group for Behçet’s Disease (1990) Criteria for diagnosis of Behçet’s disease. Lancet 335:1078–80

  12. Yazici H, Esen F (2008) Mortality in Behçet’s syndrome. Clin Exp Rheumatol 26(Suppl 51):S138–S140

    PubMed  CAS  Google Scholar 

  13. Talarico R, d'Ascanio A, Figus M, Stagnaro C, Ferrari C, Elefante E et al (2012) Behçet’s disease: features of neurological involvement in a dedicated centre in Italy. Clin Exp Rheumatol 30(3 Suppl 72):S69–S72

    PubMed  CAS  Google Scholar 

  14. Akman-Demir G, Serdaroglu P, Tasçi B (1999) Clinical patterns of neurological involvement in Behçet’s disease: evaluation of 200 patients. The Neuro-Behçet Study Group. Brain 122(Pt 11):2171–2182

    Article  PubMed  Google Scholar 

  15. Tursen U, Gurler A, Boyvat A (2003) Evaluation of clinical findings according to sex in 2313 Turkish patients with Behçet’s disease. Int J Dermatol 42:346–351

    Article  PubMed  Google Scholar 

  16. Hamuryudan V, Hatemi G, Tascilar K, Sut N, Ozyazgan Y, Seyahi E, Mat C, Yurdakul S, Yazici H (2010) Prognosis of Behcet’s syndrome among men with mucocutaneous involvement at disease onset: long-term outcome of patients enrolled in a controlled trial. Rheumatology (Oxford) 4(1):173–177

    Article  Google Scholar 

  17. Alpsoy E, Donmez L, Onder M, Gunasti S, Usta A, Karincaoglu Y, Kandi B, Buyukkara S, Keseroglu O, Uzun S, Tursen U, Seyhan M, Akman A (2007) Clinical features and natural course of Behçet’s disease in 661 cases: a multicentre study. Br J Dermatol 157(5):901–906

    Article  PubMed  CAS  Google Scholar 

  18. Kural-Seyahi E, Fresko I, Seyahi N et al (2003) The long-term mortality and morbidity of Behçet syndrome: a 2-decade outcome survey of 387 patients followed at a dedicated center. Medicine 82:60–76

    Article  PubMed  Google Scholar 

  19. Tugal-Tutkun I, Onal S, Altan-Yaycioglu R, Huseyin Altunbas H, Urgancioglu M (2004) Uveitis in Behcet disease: an analysis of 880 patients. Am J Ophthalmol 138:373–380

    Article  PubMed  Google Scholar 

  20. Alibaz-Oner F, Mumcu G, Kubilay Z, Ozen G, Celik G, Karadeniz A, Can M, Oner SY, Inanc N, Atagunduz P, Ergun T, Direskeneli H (2014) Unmet need in Behcet’s disease: most patients in routine follow-up continue to have oral ulcers. Clin Rheumatol 33(12):1773–1776

    Article  PubMed  Google Scholar 

  21. Caso F, Costa L, Rigante D, Lucherini OM, Caso P, Bascherini V, Frediani B, Cimaz R, Marrani E, Nieves-Martín L, Atteno M, Raffaele CG, Tarantino G, Galeazzi M, Punzi L, Cantarini L (2014) Biological treatments in Behçet’s disease: beyond anti-TNF therapy. Mediat Inflamm. doi:10.1155/2014/107421

  22. Ozguler Y, Hatemi G, Yazici H (2014) Management of Behçet’s syndrome. Curr Opin Rheumatol 26(3):285–291

    Article  PubMed  CAS  Google Scholar 

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Correspondence to Rosaria Talarico.

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Talarico, R., Cantarini, L., d’Ascanio, A. et al. Development of de novo major involvement during follow-up in Behçet’s syndrome. Clin Rheumatol 35, 247–250 (2016). https://doi.org/10.1007/s10067-015-2906-1

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  • DOI: https://doi.org/10.1007/s10067-015-2906-1

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