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

Rheumatology (Oxford). 2011 Jan;50(1):184-8. doi: 10.1093/rheumatology/keq324. Epub 2010 Oct 29.


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.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • 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