Registries in rheumatological and musculoskeletal conditions. Paediatric Behçet's disease: an international cohort study of 110 patients. One-year follow-up data.

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Serval ID
serval:BIB_83BD206A3B29
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Registries in rheumatological and musculoskeletal conditions. Paediatric Behçet's disease: an international cohort study of 110 patients. One-year follow-up data.
Journal
Rheumatology
Author(s)
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 T.A.
Working group(s)
PED-BD International Expert Committee
Contributor(s)
Ozen S., Ozdogan H., Gul A., Shahram F., Hofer M., Gattorno M., Koné-Paut I.
ISSN
1462-0332 (Electronic)
ISSN-L
1462-0324
Publication state
Published
Issued date
2011
Volume
50
Number
1
Pages
184-188
Language
english
Notes
Publication types: Journal Article ; Research Support, Non-U.S. Gov'tPublication Status: ppublish
Abstract
OBJECTIVE: To set-up an international cohort of patients suspected with Behçet's disease (BD). The cohort is aimed at defining an algorithm for definition of the disease in children.
METHODS: International experts have defined the inclusion criteria as follows: recurrent oral aphthosis (ROA) plus one of following-genital ulceration, erythema nodosum, folliculitis, pustulous/acneiform lesions, positive pathergy test, uveitis, venous/arterial thrombosis and family history of BD. Onset of disease is <16 years, disease duration is ≤3 years, future follow-up duration is ≥4 years and informed consent is obtained. The expert committee has classified the included patients into: definite paediatric BD (PED-BD), probable PED-BD and no PED-BD. Statistical analysis is performed to compare the three groups of patients. Centres document their patients into a single database.
RESULTS: At January 2010, 110 patients (56 males/54 females) have been included. Mean age at first symptom: 8.1 years (median 8.2 years). At inclusion, 38% had only one symptom associated with ROA, 31% had two and 31% had three or more symptoms. A total of 106 first evaluations have been done. Seventeen patients underwent the first-year evaluation, and 36 had no new symptoms, 12 had one and 9 had two. Experts have examined 48 files and classified 30 as definite and 18 as probable. Twenty-six patients classified as definite fulfilled the International Study Group criteria. Seventeen patients classified as probable did not meet the international criteria.
CONCLUSION: The expert committee has classified the majority of patients in the BD group although they presented with few symptoms independently of BD classification criteria.
Keywords
Adolescent, Age of Onset, Algorithms, Behcet Syndrome/genetics, Behcet Syndrome/physiopathology, Child, Child, Preschool, Cohort Studies, Databases, Factual, Diagnosis, Differential, Disease Progression, Female, Follow-Up Studies, Genetic Predisposition to Disease, Humans, International Cooperation, Male, Musculoskeletal Diseases/genetics, Musculoskeletal Diseases/physiopathology, Pedigree, Registries, Severity of Illness Index, Sex Factors, Statistics as Topic, Young Adult
Pubmed
Web of science
Open Access
Yes
Create date
04/01/2013 12:22
Last modification date
25/09/2019 7:09
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