Update on the Diagnosis of Behçet’s Disease

Abstract

Behçet’s disease (BD) is a systemic inflammatory disease with unknown etiology. It is characterized by recurrent mucocutaneous lesions and major organ disease such as ocular, neurologic, vascular, and gastrointestinal manifestations. The diagnosis of BD is mainly based on clinical manifestations after ruling out other potential causes. There are no specific laboratory, histopathologic, or genetic findings for the diagnosis of BD. The International Study Group (ISG) criteria set is still the most widely used set for the diagnosis. The main limitation of this criteria set is the lack of major organ manifestations such as vascular, neurologic, and gastrointestinal involvement. The ICBD 2014 criteria are more sensitive, especially in early disease. However, patients with such as spondyloarthritis can easily meet this criteria set, causing overdiagnosis. Diagnosing BD can be a big challenge in daily practice, especially in patients presenting with only major organ involvement such as posterior uveitis, neurologic, vascular, and gastrointestinal findings with or without oral ulcers. These patients do not meet ISG criteria and can be diagnosed with “expert opinion” in countries with high BD prevalence. The pathergy test is the only diagnostic test used as diagnostic or classification criteria for BD. Our recent studies showed that common femoral vein (CFV) thickness measurement can be a valuable, practical, and cheap diagnostic tool for BD with sensitivity and specificities higher than 80% for the cut-off value of 0.5 mm. However, the diagnostic accuracy of CFV measurement should be investigated in other disease groups in the differential diagnosis of BD and in also different ethnic populations.