Behçet’s disease is a rare multi-systemic inflammatory disease with unknown etiology which involves principally oral and genital mucosa, skin and eyes. Average age at onset of the disease is about 25-30 years, but it may be diagnosed before the age of 16. It is not very rare in Italy, even though there are limited data concerning epidemiology. Aim of this study is to describe the baseline data of an Italian cohort of patients with as having BD or probable BD.
We described the baseline data of the first national epidemiological study on children coming from 16 Italian Pediatric Rheumatologic Centers diagnosed by the treating physicians as having Behçet’s Disease. Data on demographic characteristics, clinical features and therapy were collected. We then compared our findings to those of international pediatric cohort studies and also retrospectively evaluated the ability to diagnose BD using ISG, ICBD and, for the first time, the new PEDBD criteria.
The study included 110 patients (62 M, 48F). Average age at onset was 8.34±4.11 years. The frequencies of signs/symptoms were: recurrent oral aphtosis 94.5%, genital ulcers 33.6%, ocular 43.6%, gastrointestinal 42.7%, musculoskeletal 42.7%, neurological 30.9% and vascular involvement 10%. Thirty-two patients (29.1%) fulfilled ISG, 78 (70.9%) ICBD, 50 (45.5%) PEDBD criteria and 31 (28%) didn’t fulfill any of them. The most frequently used treatments were colchicine and corticosteroids followed by immunosuppressants. Four patients received biologic therapy (anti TNF-α and anti-IL-1) to treat severe organ involvement.
Recurrent oral aphtosis was the most frequent clinical manifestation, followed by ocular involvement. Gastrointestinal lesions were more frequent in Italy than in non-European countries as opposed to genital ulcers. Skin, ocular and vascular manifestations had a higher frequency in males and genital ulcers in females. Constitutional symptoms were present in 44.5% and recurrent fever in one third of our population.
Caso F, Costa L, Rigante D, et al. Biological treatments in Behçet's disease: beyond anti-TNF therapy. Mediat Inflamm. 2014;2014:107421. Epub 2014 Jun 30 CrossRef
Olivieri I, Leccese P, Padula A, Nigro A, Palazzi C, Gilio M, D'Angelo S. High prevalence of Behçet’s disease in southern Italy. Clin Exp Rheumatol. 2013;31(3 Suppl 77):28–31. PubMed
Criteria for diagnosis of Behçet's disease. International study Group for Behçet's disease. Lancet. 1990;335(8697):1078–80.
International Team for the Revision of the International Criteria for Behçet's Disease (ITR-ICBD). The international criteria for Behçet's disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. Eur Acad Dermatol Venereol. 2014;28(3):338–47. CrossRef
Koné-Paut I, Shahram F, Darce-Bello M, Cantarini L, Cimaz R, Gattorno M, Anton J, Hofer M, Chkirate B, Bouayed K, Tugal-Tutkun I, Kuemmerle-Deschner J, Agostini H, Federici S, Arnoux A, Piedvache C, Ozen S, PEDBD group. Consensus classification criteria for paediatric Behçet's disease from a prospective observational cohort: PEDBD. Ann Rheum Dis. 2016;75(6):958–64. CrossRefPubMed
Batu ED, Sönmez HE, Sözeri B, Butbul Aviel Y, Bilginer Y, Özen S. The performance of different classification criteria in paediatric Behçet's disease. Clin Exp Rheumatol. 2017;Suppl 108(6):119–123. Epub 2017 Apr 5.
Hamzaoui A, Jaziri F, Ben Salem T, Said Imed Ben Ghorbel F, Lamloum M, Smiti Khanfir M, Houman Mohamed H. Comparison of clinical features of Behcet disease according to age in a Tunisian cohort. Acta Med Iran. 2014;52(10):748–51. PubMed
Karincaoglu Y, Borlu M, Toker SC, Akman A, Onder M, Gunasti S, Usta A, Kandi B, Durusoy C, Seyhan M, Utas S, Saricaoglu H, Ozden MG, Uzun S, Tursen U, Cicek D, Donmez L, Alpsoy E. Demographic and clinical properties of juvenile-onset Behçet's disease: a controlled multicenter study. J Am Acad Dermatol. 2008;58(4):579–84. Epub 2007 Nov 28 CrossRefPubMed
Emmi G, Talarico R, Lopalco G, Cimaz R, Cantini F, Viapiana O, Olivieri I, Goldoni M, Vitale A, Silvestri E, Prisco D, Lapadula G, Galeazzi M, Iannone F, Cantarini L. Efficacy and safety profile of anti-interleukin-1 treatment in Behçet's disease: a multicenter retrospective study. Clin Rheumatol. 2016;35(5):1281–6. CrossRefPubMed
Pagnini I, Bondi T, Simonini G, Giani T, Marino A, Cimaz R. Successful treatment with canakinumab of a paediatric patient with resistant Behçet's disease. Rheumatology (Oxford). 2015;54(7):1327–8. CrossRef
- A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry
Maria Cristina Maggio
Alma Nunzia Olivieri
- BioMed Central
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