Behcet’s Syndrome International Study Group Criteria

Behcet's disease is an uncommon inflammatory disease for which there are several proposed diagnostic criteria. At the time of the International Study Group for Behcet's disease proposed diagnostic criteria (Lancet 1990), there were already five commonly used criteria which has since significantly expanded (Davatchi 2011). This study group sought to unify common disease features to assist in research and improve clinician diagnostic capability. The sensitivity of the ISG 1990 criteria is 92% with a specificity 97% making it an effective tool to rule in cases. It has been suggested that given the low prevalence of Behcet's disease, a more sensitive criteria ought to be adopted. The International Criteria for Behcet's Disease (ICBD) was created in 2006 and has a more sensitive but less specific tool to mitigate this issue. They both continue to be used in clinical practice and research studies, among others.

Variable & Associated Points

Required Criteria

• Recurrent oral ulcerations observed by physician or patient, occurring ≥ 3 times in 1 year [Yes / No]
• These can be minor or major aphthous ulcers or herpetiform ulcers

Minor Criteria

• Recurrent genital ulceration (aphthous or scarring) observed by physician or patient [yes/no]
• Eye lesions observed by physician [yes / no]
• Anterior uveitis
• Posterior uveitis
• Cells in vitreous on slit lamp exam
• Retinal vasculitis observed by ophthalmologist
• Skin lesions present [yes / no]
• Erythema nodosum (patient or physician observed)
• Pseudo folliculitis (observed by physician)
• Papulopustular lesions (observed by physician)
• Acneiform nodules (observed by physician in post-adolescent patients not on steroids)
• Positive pathergy (Behcetine) test read by physician within 24-28 hours [yes / no]