BehçEt’s Disease in Children: Eastern Mediterranean Experience

Hafize Emine Sonmez¹, Ezgi Deniz Batu¹, Betul Sozeri², Yonatan Butbul Aviel³, Yelda Bilginer⁴ and Seza Ozen⁵, ¹Pediatric Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey, ²Pediatric Rheumatology, Erciyes University Faculty of Medicine, Kayseri, Turkey, ³Pediatric Rheumatology, Rambam Medical Center, Haifa, Israel, ⁴Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, ANKARA, Turkey, ⁵Department of Pediatrics, Division of Rheumatology, Hacettepe University Faculty of Medicine, Ankara, Turkey

Meeting: 2016 ACR/ARHP Annual Meeting

Date of first publication: September 28, 2016

Keywords: Behcet's syndrome, Diagnostic criteria, Outcome measures, pediatrics and vasculitis

Session Information

Date: Tuesday, November 15, 2016

Title: Vasculitis - Poster III: Rarer Vasculitides

Session Type: ACR Poster Session C

Session Time: 9:00AM-11:00AM

Background/Purpose: Behçet’s disease (BD) is a variable vessel vasculitis which is more common in adults. The most widely used diagnostic criteria for adult onset disease is the International Behçet’s Study Group (ISG) criteria. A new set of criteria for the classification of Behçet’s disease (BD) in children (the pediatric BD [PEDBD] criteria) has been proposed recently. To evaluate the disease activity; there are mainly two severity scores the BD dynamic activity measure (IBDDAM) and BD current activity form (BDCAF). We aimed to test the performance of the PEDBD criteria compared to the ISG criteria and to check the correlation between the severity score systems and physician global assessment (PGA) in pediatric BD patients.

Methods: Two centers from Turkey and one center from Israel participated in this study. The disease onset was ≤16 years of age. As controls, pediatric patients with rheumatologic other diseases.

Results: Sixty-eight BD (44.1% male) patients and 93 control patients were included. The sensitivity and specificity of the PEDBD and the ISG criteria were 73.5%/52.9% and 98.9%/100%, respectively. Thirty-two (47%) patients with BD failed to fulfill the ISG criteria. However, almost all of these patients met the PEDBD criteria. The median (min-max) IBDDAM and BDCAF scores at diagnosis were 6 (1-23) and 4 (1-7) and significantly decreased to 1 (0-8) and 1 (0-4) respectively at latest follow-up (p<0.001 for both). The median (min-max) PGA score at diagnosis was 5 (2-9) and significantly decreased to 1 (0-7) at latest follow-up (p<0.001). IBDDAM had a strong positive correlation 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).

Conclusion: In our pediatric series, the PEDBD criteria showed better sensitivity than the ISG criteria and the specificities were close. The higher sensitivity is a big advantage for pediatric patients since early diagnosis is very important. Our study also demonstrated that the severity scores were positively correlated with each other and PGA and thus may be used in clinical practice to evaluate children with BD.

Disclosure: H. E. Sonmez, None; E. D. Batu, None; B. Sozeri, None; Y. Butbul Aviel, None; Y. Bilginer, None; S. Ozen, None.

To cite this abstract in AMA style:

Sonmez HE, Batu ED, Sozeri B, Butbul Aviel Y, Bilginer Y, Ozen S. BehçEt’s Disease in Children: Eastern Mediterranean Experience [abstract]. Arthritis Rheumatol. 2016; 68 (suppl 10). https://acrabstracts.org/abstract/behcets-disease-in-children-eastern-mediterranean-experience/. Accessed .

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