Zeitschrift für medizinische und chirurgische Pathologie
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Behcet’s Disease Criteria

Fereydoun Davatchi, Bahar Sadeghi Abdollahi, Farhad Shahram, Cheyda Chams Davatchi, Abdolhadi Nadji, Hormoz Shams, Massoomeh Akhlaghi, Tahreh Faezi, Zahra Ghodsi, Farimah Ashofteh, Negin Mohtasham, Hoda Kavosi, Mariam Masoumi

Objective: From 1946 to 2014, 16 different sets of Classification/Diagnosis criteria, from different countries, have been made for Behcet’s Disease. Among them, there are two International criteria sets, the International Study Group (ISG) criteria in 1990 by the collaboration of 7 countries, and the International Criteria for Behcet’s Disease (ICBD) in 2010 by the collaboration of 27 countries. The aim of this study is to compare the performance of them, in new patients and controls, from the Iran registry of Behcet’s Disease.
Methods: Patients (1323) and controls (2438) are consecutive patients, seen from 2010 to 2016. The diagnosis was clinical and by expert opinion. Sensitivity, specificity, accuracy, optimization, efficiency, positive predictive value, negative predictive value, positive likelihood ratio, negative likelihood ratio, diagnostic odds ratio, and Youden’s index were calculated.
Results: The sensitivity of ISG was 64% versus 96.9% ICBD (a difference of 32.9%), while specificity was 99.9% versus 97.4% (a difference of 2.5%), and accuracy 87.3% vs. 97.2% (a difference of 9.9%). The optimization (100- difference between sensitivity and specificity. The best score is 100) was 64.1 versus 99.7 (a difference of 35.6). The efficiency ((optimization/100) × accuracy. The best score is 100) was 56 versus 96.9 (a difference of 40.9).
Conclusion: The differences for all the items was statistically highly significant, even for the specificity, although numerically the difference was small, just 2.5%. However, clinically, the difference was not relevant. The performance of ICBD is much higher than that of ISG. The ICBD criteria have a much higher sensitivity, accuracy, optimization, and efficiency, with clinically, approximately the same specificity.