Skip to main page content
U.S. flag

An official website of the United States government

Dot gov

The .gov means it’s official.
Federal government websites often end in .gov or .mil. Before sharing sensitive information, make sure you’re on a federal government site.

Https

The site is secure.
The https:// ensures that you are connecting to the official website and that any information you provide is encrypted and transmitted securely.

Access keys NCBI Homepage MyNCBI Homepage Main Content Main Navigation
. 2017 Dec 21;15(1):84.
doi: 10.1186/s12969-017-0213-x.

A national cohort study on pediatric Behçet's disease: cross-sectional data from an Italian registry

Romina Gallizzi  1 , Caterina Pidone  2 , Luca Cantarini  3 , Martina Finetti  4 , Marco Cattalini  5 , Giovanni Filocamo  6 , Antonella Insalaco  7 , Donato Rigante  8 , Rita Consolini  9 , Maria Cristina Maggio  10 , Adele Civino  11 , Silvana Martino  12 , Alma Nunzia Olivieri  13 , Giovanna Fabio  14 , Serena Pastore  15 , Angela Mauro  16 , Diana Sutera  2 , Giuseppe Trimarchi  17 , Nicolino Ruperto  18 , Marco Gattorno  18 , Rolando Cimaz  16
Affiliations

A national cohort study on pediatric Behçet's disease: cross-sectional data from an Italian registry

Romina Gallizzi et al. Pediatr Rheumatol Online J. .

Erratum in

  • Correction to: A national cohort study on pediatric Behçet's disease: cross-sectional data from an Italian registry.
    Gallizzi R, Pidone C, Cantarini L, Finetti M, Cattalini M, Filocamo G, Insalaco A, Rigante D, Consolini R, Maggio MC, Civino A, Martino S, Olivieri AN, Fabio G, Pastore S, Mauro A, Sutera D, Trimarchi G, Ruperto N, Gattorno M, Cimaz R; for EUROFEVER and the Paediatric Rheumatology International Trials Organisation (PRINTO). Gallizzi R, et al. Pediatr Rheumatol Online J. 2018 Apr 23;16(1):29. doi: 10.1186/s12969-018-0241-1. Pediatr Rheumatol Online J. 2018. PMID: 29685142 Free PMC article.

Abstract

Background: 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.

Methods: 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.

Results: 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.

Conclusions: 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.

Keywords: Behçet’s disease; Children; Clinical features; Diagnostic criteria; Treatment.

PubMed Disclaimer

Conflict of interest statement

Ethics approval and consent to participate

Not applicable.

Consent for publication

Not applicable.

Competing interests

The authors declare that they have no competing interests.

Publisher’s Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Figures

Fig. 1
Fig. 1
Italian Pediatric Rheumatologic Centers
Fig. 2
Fig. 2
Frequency of clinical features
See this image and copyright information in PMC

References

    1. Ciccarelli F, De Martinis M, Ginaldi L. An update on autoinflammatory diseases. Curr Med Chem. 2014;21(3):261–269. doi: 10.2174/09298673113206660303. - DOI - PMC - PubMed
    1. Kalayciyan A, Zouboulis C. An update on Behçet's disease. J Eur Acad Dermatol Venereol. 2007;21(1):1–10. doi: 10.1111/j.1468-3083.2006.01863.x. - DOI - PubMed
    1. Caso F, Costa L, Rigante D, et al. Biological treatments in Behçet's disease: beyond anti-TNF therapy. Mediat Inflamm. 2014;2014:107421. doi: 10.1155/2014/107421. - DOI - PMC - PubMed
    1. Mendes D, Correia M, Barbedo M, Vaio T, Mota M, Gonçalves O, Valente J. Behçet's disease-a contemporary review. J Autoimmun. 2009;32(3-4):178–188. doi: 10.1016/j.jaut.2009.02.011. - DOI - PubMed
    1. Davatchi F, Shahram F, Chams-Davatchi C, Shams H, Nadji A, Akhlaghi M, Faezi T, Ghodsi Z, Faridar A, Ashofteh F, Sadeghi Abdollahi B. Behcet’s disease: from east to west. Clin Rheumatol. 2010;29(8):823–833. doi: 10.1007/s10067-010-1430-6. - DOI - PubMed