Pediatric rheumatology

Validation of the PEDiatric Behçet’s Disease classification criteria: an evidence-based approach

Rheumatology (Oxford)Published Diagnostic accuracy study

An erratum to this article was published (Rheumatology (Oxford), 1 Dec 2024 ↗)

Authors' conclusion

The PEDBD criteria were very specific, while the ICBD were more sensitive.

Study context

Population
Pediatric patients with Behçet's disease (BD) and confounding autoinflammatory diseases randomly selected from the Eurofever Registry70 BD
Participants
210 patients
Comparison
PEDBD vs ISG vs ICBD classification criteria
Outcome
Sensitivity, specificity and accuracy of the criteria vs table consensus
Methodological detailsevidence-based validation, clinicians blinded to original diagnosis, table consensus as gold standard (agreement ≥ 80%), 11 experienced clinicians/researchers blinded to the original diagnosis evaluated the patients
  • Evidence-based validation
  • Clinicians blinded to original diagnosis
  • Table consensus as gold standard (agreement ≥ 80%)
  • 11 experienced clinicians/researchers blinded to the original diagnosis evaluated the patients

Performance

As reported in the abstract

Performance in confirmed BD vs confounding diseases
Test or modelSensitivitySpecificityAccuracy
ISG0.501.000.91
ICBD0.790.970.94
PEDBD0.580.990.92

Sensitivity

ISG0.50
ICBD0.79
PEDBD0.58

Specificity

ISG1.00
ICBD0.97
PEDBD0.99

Accuracy

ISG0.91
ICBD0.94
PEDBD0.92

Further findings

Expert consensus reached · end of third round

Consensus reached
139/210 patients (66.2%)
Confirmed BD (consensus ≥80%)
24
Probable BD (agreement 60-79%)
10

BD distinctive elements vs confounding diseases

Older age at onset, oral and genital ulcers, skin papulo-pustular lesions, positive pathergy test and posterior uveitis were BD distinctive elements

Original publication

Validation of the PEDiatric Behçet’s Disease classification criteria: an evidence-based approach. Rheumatology (Oxford). Published 22 November 2023. doi:10.1093/rheumatology/kead609

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