Abstract

BackgroundBehçet's disease (BD) is characterized by recurrent oral and/or genital aphthous ulcers accompanied by cutaneous, ocular, articular, gastrointestinal and central nervous system lesions. Diagnosing BD can be a clinical challenge in patients presenting with a limited number of organ manifestations, especially with single major organ involvement. We reported the first controlled doppler ultrasound study showing increased common femoral vein (CFV) thickness in BD (1). We recently also showed that increased CFV thickness is a distinctive feature of BD, rarely present in other inflammatory or vascular diseases such as ankylosing spondylitis, systemic vasculitides, venous insufficiency, and non-inflammatory DVT with a specificity higher than 80% for the cut-off value of ≥ 0.5 mm. We suggest that CFV thickness measurement is an easy, non-invasive diagnostic test for BD (2).ObjectivesIn this study, we aimed to assess the diagnostic performance of CFV thickness measurement in patients with ‘Incomplete’ Behçet’s Disease diagnosed by expert opinion.MethodsWe included 28 patients with incomplete BD (15 male, 12 female) diagnosed with expert opinion and followed in the Marmara University Behcet’s Clinic. Demographic, clinical characteristics and treatment data were recorded during routine visits. Common femoral vein wall thickness was measured by an experienced radiologist at the same day.ResultsMedian age was 34.3 years and median disease duration 2 years (0-16). Four patients were newly diagnosed. At follow-up onset, oral ulcers were present in 22 (78.6%), genital ulcers in 6 (21.6%), papulopustular lesions in 4 (14.3%) and pathergy positivity in 5 (17.9%) patients. Ten (35.7%) patients had familial BD. While 24 (85.7%) patients had major organ involvement, 4 (14.3%) patients had mucocutaneous disease. Distrubution of major organ involvements were given in Figure 1.Figure 1.Distribution of major organ involvements (n) in study groupAll patients except 1, had CFV thickness value above the cut-off value of ≥0.5 mm. Right CFV thickness was 0.71 (0.3-1.3) mm and left CFV thickness 0.72 (0.4-1.2) mm. Bilateral femoral vein thicknesses were similar in patients with and without an history of familial BD.ConclusionDiagnosing BD can be challenging in patients presenting with one major organ involvement such as oral ulcers and posterior uveitis, brain-stem disease or arterial aneurysms, especially in countries with a low prevalence. These patients are generally diagnosed as ‘incomplete’ BD by ‘expert opinion’. Early diagnosis is of utmost importance in some of these cases, especially with venous thrombosis as their management differs from non-inflammatory venous thrombosis, necessitating immunosuppressive use rather than anticoagulant therapy. Our results show that CFV thickness measurement with Doppler US, a non-invasive, fast and cost-effective radiological modality, is a valuable diagnostic test in incomplete BD, especially with major organ involvement.

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