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Ophthalmologic Manifestations of Behcet Disease


Behçet disease is a systemic disorder characterized by recurrent aphthous ulcers and intraocular inflammation. The clinical triad of uveitis with recurrent oral and genital ulcers bears the name of Hulusi Behçet, a Turkish dermatologist who described 3 patients who had this triad.

The Behçet’s Disease Research Committee of the Ministry of Health and Welfare of Japan first proposed formal diagnostic criteria in 1972. This set of criteria, which has been used throughout the world, classifies disease findings into 4 major criteria and 5 minor criteria. When all 4 major criteria are met, the disease is said to be of the complete type, whereas the incomplete type consists of various combinations of major and minor criteria, with added weight given to ocular disease.

In 1990, The International Study Group (ISG) for Behçet’s Disease proposed a separate set of diagnostic criteria for Behçet disease. Based on these criteria, a diagnosis of Behçet disease requires recurrent oral ulceration and at least 2 additional criteria, including recurrent genital ulcers, ocular lesions, skin lesions, and a positive pathergy test. The ISG criteria for Behçet disease have excellent specificity, but lack sensitivity. The International Criteria for Behçet’s Disease (ICBD) was created in 2006, as a replacement to the ISG criteria. For ICBD, vascular lesions were added, while oral aphthosis is no longer mandatory. Accruing 3 or more points is an indication of the diagnosis of Behçet disease (genital aphthosis, 2 points; eye lesions, 2 points; and the remaining each are 1 point).

A recent study compared the performance of the ISG and ICBD criteria. Their sensitivity, specificity, and accuracy (percent agreement), were tested in 3 independent cohorts of patients from the Far East (China), Middle East (Iran), and Europe (Germany). The sensitivity for ISG criteria was, respectively, 65.4%, 78.1%, and 83.7%, and, for ICBD, was 87%, 98.2%, and 96.5%. The specificity for ISG was, respectively, 99.2%, 98.8%, and 89.5%, and, for ICBD was 94.1%, 95.6%, and 73.7%. The accuracy for ISG was 74.2%, 85.5%, and 85.5%, and, for ICBD was 88.9%, 97.3%, and 89.5%. The conclusion is that ICBD has better sensitivity and accuracy than ISG.

Other Medscape Reference articles on Behçet disease include Behcet disease (Dermatology) and Behcet disease (Rheumatology).

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