Background/Purpose:

ICBD is the new international criteria for diagnosis/classification of Behcet’s Disease, created in 2006. The scoring system for ICBD depends on 2 points for ocular lesions and genital ulcers each, and 1 point for each of oral aphtosis; skin manifestations; vascular manifestations; and positive-pathergy test. Three or more points are required to consider the patient as a case of Behcet’s Disease. The validity of the criteria has been confirmed for Iranian patients in 2010. To determine the performance of ICBD in Iranian children with Behcet’s Disease (Diagnosed before the age of 16), the sensitivity of ICBD is compared with 4 most commonly used diagnosis/classification criteria (Revised Japan, O’Duffy, International Study Group [ISG] and Classification Tree).

Methods:

According to the data registry for Behcet’s Disease patients in Rheumatology Research Center, Tehran University of Medical Sciences, Iran, all the patients diagnosed before the age of 16 (during 1975-2011) are included in the study. The fulfillment of each of the 5 criteria is evaluated for each patient and the sensitivity of different sets of criteria in childhood BD is calculated.

Results:

180 children out of 6813 BD patients (2.64 %, CI: 0.4) are selected (86 male and 94 female). The mean age was 10.7 years (SD: 3.4, CI: 0.5), mean duration 8.2 years (SD: 6.7), mean follow up 5 years (SD: 6.2) and the diagnosis delay 3.2 years (SD: 2.7).

I) 174 patients fulfilled the ICBD criteria (sensitivity 96.66%); 171 patients met Classification Tree criteria (sensitivity 95%); 156 patients for Revised Japan criteria (sensitivity 86.66%); 126 patients met ISG criteria (sensitivity 70%) and 103 patients fulfilled O’Duffy criteria with sensitivity of 57.2%.

II) Although the majority of the patients met all 5 sets of criteria, some of the patients could fulfill different combinations of criteria sets as described below;

88 patients (48.88%) met all 5 criteria; 30 patients (16.66%) fulfilled ICBD, Japan and Classification Tree; 26 patients (14.44%) met all the assessed criteria except for O’Duffy ; 7 patients (3.9%)  met all the assessed criteria except for ISG;  6 patients (3.33%)  could meet only ICBD and Classification Tree ; 6 patients (3.33%) met ICBD, Classification Tree and ISG;  5 patients (2.77%) met all the assessed criteria except for Japan; 3 patients (1.66 %) fulfilled ICBD and Japan Criteria;  2 patients (1.1 %) fulfilled ICBD, O’Duffy and Classification Tree; 2 patients (1.1 %) fulfilled ICBD, Japan and O’Duffy; 1 patient (1.1%) met ISG and Classification Tree and  4 patients (2.22 %) could not meet any of the 5 assessed criteria.

Conclusion:

The most sensitive criteria for Iranian children with Behcet’s disease was ICBD, followed by Classification Tree, Japan and ISG and O’Duffy. The higher sensitivity in ICBD, Classification Tree and Japan criteria may be explained by the prominence of the eye involvement in children and the importance of ocular lesions in these criteria sets.

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ACR Meeting Abstracts - https://acrabstracts.org/abstract/performance-of-icbd-international-criteria-for-behcets-disease-in-iranian-children-with-behcets-disease/