Comparison of the Performance of Three Classification Criteria for Behcet's Disease in a Single Centre Pediatric Cohort from South India

Background: A new International Paediatric criteria for Behcet's disease (PEDBD) 2015 has been introduced in the presence of existing revised International Criteria for Behcet's Disease (ICBD) and the International Study Group (ISG) criteria. In this study, we compared the performance of the new PEDBD criteria with the existing criteria in the pediatric group of Behcet's disease. Methods: In this retrospective study eligible patients were identified from a database and their already recorded characteristics were analyzed. A total of 25 patients who visited the pediatric rheumatology clinic were included in the cohort. Performance of the ISG, revised ICBD, and PEDBD 2015 was evaluated in terms of sensitivity, specificity, positive predictive value (PPV), negative predictive value (NPV), negative likelihood ratio (NLR), positive likelihood ratio (PLR), diagnostic odds ratio (DOR), accuracy, and Youden's index (YI). Results: The revised ICBD has the maximum sensitivity (88.24%) when compared to PEDBD (64.71%) and ISG (47.05%). ISG had 100% specificity, with a comparable value in revised ICBD (87.5%) and PEDBD (87.5%). PPV was similar in all the three criteria with ISG 100%, revised ICBD 93.75%, and PEDBD 91.67%. NPV in revised ICBD was 77.78%, ISG was 47.06%, and PEDBD 53.85%. PLR of revised ICBD was 7.06 and PEDBD was 5.18. NLR was detected to be 0.53 in ISG as it was 0.40 in PEDBD and 0.13 in revised ICBD. The DOR in revised ICBD was 52 and PEDBD was 12.83. YI was 0.47 in ISG, 0.76 in revised ICBD, and 0.52 in PEDBD. The accuracy was best for revised ICBD 88% and PEDBD 72%. Conclusion: Revised ICBD criteria hold a higher statistical significance in the diagnosis of the Behcet's disease even in the pediatric age group. PEDBD has a comparable sensitivity and PPV although the NPV and specificity are very low in comparison to the other criteria. It was observed that oral aphthosis, despite being the most common manifestation in the Behcet's disease pediatric group, other features, mainly vascular involvement was the most common presenting complaint.