A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry
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 I...
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doaj-3b948eb254084c51838cd6dec7d52f722020-11-24T22:52:54ZengBMCPediatric Rheumatology Online Journal1546-00962017-12-011511710.1186/s12969-017-0213-xA national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registryRomina Gallizzi0Caterina Pidone1Luca Cantarini2Martina Finetti3Marco Cattalini4Giovanni Filocamo5Antonella Insalaco6Donato Rigante7Rita Consolini8Maria Cristina Maggio9Adele Civino10Silvana Martino11Alma Nunzia Olivieri12Giovanna Fabio13Serena Pastore14Angela Mauro15Diana Sutera16Giuseppe Trimarchi17Nicolino Ruperto18Marco Gattorno19Rolando Cimaz20Unit of Pediatrics, Department of Human Pathology in Adulthood and Childhood “G. Barresi”, University of MessinaUnit of Pediatrics, Department of Human Pathology in Adulthood and Childhood “G. Barresi”, University of MessinaRheumatology Unit Policlinico “Le Scotte”, University of SienaUnit of Pediatrics II, Gaslini InstitutePediatric Clinic University of Brescia and Spedali Civili of BresciaPediatric Rheumatology, Fondazione IRCCS Ca’ Grande, Ospedale MaggioreDepartment of Pediatric Medicine, Division of Rheumatology, Bambino Gesù Children’s HospitalInstitute of Pediatrics, Università Cattolica Sacro Cuore, Fondazione Policlinico Universitario, “A. Gemelli”Unit of Pediatrics, A.O.UOspedale dei Bambini “G. Di CristinaAzienda Ospedaliera Card. G. PanicoUnit of Pediatrics, Ospedale Regina MargheritaSecond University Of Study of NapoliFondazione IRCCS Ca’ Grande Ospedale MaggioreIRCCS Burlo GarofaloPediatric Rheumatology Unit, AOU Meyer, University of FlorenceUnit of Pediatrics, Department of Human Pathology in Adulthood and Childhood “G. Barresi”, University of MessinaUniversità di Messina Dipartimento di Economia MessinaInstitute “G. Gaslini”, UO Pediatria IIInstitute “G. Gaslini”, UO Pediatria IIPediatric Rheumatology Unit, AOU Meyer, University of FlorenceAbstract 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.http://link.springer.com/article/10.1186/s12969-017-0213-xBehçet’s diseaseChildrenClinical featuresDiagnostic criteriaTreatment |
collection |
DOAJ |
language |
English |
format |
Article |
sources |
DOAJ |
author |
Romina Gallizzi Caterina Pidone Luca Cantarini Martina Finetti Marco Cattalini Giovanni Filocamo Antonella Insalaco Donato Rigante Rita Consolini Maria Cristina Maggio Adele Civino Silvana Martino Alma Nunzia Olivieri Giovanna Fabio Serena Pastore Angela Mauro Diana Sutera Giuseppe Trimarchi Nicolino Ruperto Marco Gattorno Rolando Cimaz |
spellingShingle |
Romina Gallizzi Caterina Pidone Luca Cantarini Martina Finetti Marco Cattalini Giovanni Filocamo Antonella Insalaco Donato Rigante Rita Consolini Maria Cristina Maggio Adele Civino Silvana Martino Alma Nunzia Olivieri Giovanna Fabio Serena Pastore Angela Mauro Diana Sutera Giuseppe Trimarchi Nicolino Ruperto Marco Gattorno Rolando Cimaz A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry Pediatric Rheumatology Online Journal Behçet’s disease Children Clinical features Diagnostic criteria Treatment |
author_facet |
Romina Gallizzi Caterina Pidone Luca Cantarini Martina Finetti Marco Cattalini Giovanni Filocamo Antonella Insalaco Donato Rigante Rita Consolini Maria Cristina Maggio Adele Civino Silvana Martino Alma Nunzia Olivieri Giovanna Fabio Serena Pastore Angela Mauro Diana Sutera Giuseppe Trimarchi Nicolino Ruperto Marco Gattorno Rolando Cimaz |
author_sort |
Romina Gallizzi |
title |
A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry |
title_short |
A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry |
title_full |
A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry |
title_fullStr |
A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry |
title_full_unstemmed |
A national cohort study on pediatric Behçet’s disease: cross-sectional data from an Italian registry |
title_sort |
national cohort study on pediatric behçet’s disease: cross-sectional data from an italian registry |
publisher |
BMC |
series |
Pediatric Rheumatology Online Journal |
issn |
1546-0096 |
publishDate |
2017-12-01 |
description |
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. |
topic |
Behçet’s disease Children Clinical features Diagnostic criteria Treatment |
url |
http://link.springer.com/article/10.1186/s12969-017-0213-x |
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