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The performance of different classification criteria in paediatric Behçet's disease
Ezgi Deniz Batu1, Hafize Emine Sönmez1, Betul Sözeri2
1Department of Paediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey.
Insights
The new pediatric Behçet
Area of Science:
- Pediatric rheumatology
- Vasculitis research
- Disease classification and activity scoring
Background:
- Behçet's disease (BD) is a systemic vasculitis affecting various blood vessels.
- Current adult classification relies on the International Study Group (ISG) criteria.
- Pediatric BD (PEDBD) classification criteria and disease activity scores (IBDDAM, BDCAF) have been developed for children.
Purpose of the Study:
- To evaluate the performance of the PEDBD and ISG classification criteria in children with BD.
- To assess the correlation between disease activity scores (IBDDAM, BDCAF) and physician global assessment (PGA) in pediatric BD patients.
Main Methods:
- Retrospective study including 68 pediatric BD patients and 90 controls from Turkey and Israel.
- Sensitivity and specificity of PEDBD and ISG criteria were calculated.
- Disease activity was assessed using IBDDAM, BDCAF, and PGA at diagnosis and follow-up.
Main Results:
- PEDBD criteria demonstrated higher sensitivity (73.5%) compared to ISG criteria (52.9%) in pediatric BD.
- Nearly all pediatric BD patients met PEDBD criteria, while 47% did not meet ISG criteria.
- IBDDAM, BDCAF, and PGA scores significantly decreased from diagnosis to follow-up, showing positive correlations with each other.
Conclusions:
- The PEDBD criteria offer improved sensitivity for early diagnosis of pediatric BD.
- Disease activity scores (IBDDAM, BDCAF) and PGA are reliable tools for monitoring disease activity in children with BD.
Objectives:
Behçet's disease (BD) is a variable vessel vasculitis. The most widely used classification criteria for adults is the International Behçet's Study Group (ISG) criteria. Recently, the paediatric BD (PEDBD) classification criteria has been developed for children. For disease activity, there are mainly two severity scores; the Iranian BD dynamic activity measure (IBDDAM) and BD current activity form (BDCAF). We tested the performances of PEDBD and ISG criteria and the correlation between severity scores and physician global assessment (PGA) in children with BD.
Methods:
Thirty BD patients from Hacettepe University, Ankara, Turkey; 24 from Erciyes University, Kayseri, Turkey; and 14 BD patients from Rambam Medical Centre, Haifa, Israel were included. As controls, children with systemic lupus erythematosus, polyarteritis nodosa, and Crohn disease from Turkey and Israel were included. The sensitivity and specificity of the PEDBD and ISG criteria were evaluated based on the features of the patients before or at 16 years of age. The gold standard for the diagnosis of BD was based on expert opinion at each centre. Expert PGA (visual analogue scale between 0-10; where 0 indicates no disease activity), IBDDAM, and BDCAF were evaluated at the time of diagnosis and at last follow-up in all patients.
Results:
Sixty-eight BD (disease onset≤16 years; 44.1% male) and 90 control patients were included. The sensitivity and specificity of PEDBD/ISG criteria were 73.5%/52.9% and 97.7%/100%, respectively. Thirty-two (47%) patients with BD failed to fulfill ISG criteria while almost all met PEDBD criteria. The median (interquartile range; IQR) IBDDAM and BDCAF scores at diagnosis were 6(4)/4(2); significantly decreased to 1(2)/1(2), respectively at latest follow-up (p<0.001 for both). The median (IQR) PGA score at diagnosis was 5(2); significantly decreased to 1(2) at latest follow-up (p<0.001). IBDDAM positively correlated with BDCAF (r=0.637; p<0.001). PGA positively correlated with BDCAF and IBDDAM (r=0.502; p<0.001 and r=0.624;p<0.001, respectively).
Conclusions:
In our study, the PEDBD criteria showed better sensitivity than ISG criteria which is a big advantage for paediatric patients for early diagnosis. We also demonstrated that the severity scores were positively correlated with each other and PGA; thus may be used in clinical practice for paediatric BD patients.