Paediatric Behcet's disease: an international cohort study of 110 patients. One-year follow-up data

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Registries in rheumatological and musculoskeletal conditions. Paediatric

Behcet's disease: an international cohort study of 110 patients. One-year

follow-up data

http://rheumatology.oxfordjournals.org/content/early/2010/10/28/rheumatology.keq\

324.abstract

Correspondence to: Isabelle Kone-Paut, Service de Pediatrie Geneeale,

Rhumatologie Pediatrique, Centre de Reference des Maladies

Auto-inflammatoires, CHU de Bicetre, 78 rue du Geneeal Leclerc, 94270 le

Kremlin Biceere, France. E-mail: isabelle.kone-paut@...

Abstract

Objective. To set-up an international cohort of patients suspected with

Behcet'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.