Behçet's disease is a systemic vasculitis of unknown etiology characterized by recurrent oral and genital ulcers and uveitis. The vascular involvement of Behçet's disease affects arteries, veins, and blood vessels of all sizes, and it can include venous or arterial thrombosis and arterial aneurysms. There are only a few reports of an aortic aneurysm invading a vertebral body in a patient with Behçet's disease. Here, we report the case of a 45-year-old man who was initially diagnosed with vertebral invasion of a mycotic aneurysm. He underwent vascular surgery and received empirical antibiotics, but all cultures were negative. However, he had persistent, recurrent deep vein thrombosis and elevated inflammatory markers. After reviewing the pathology, a final diagnosis of Behçet's disease was made. He was successfully treated with corticosteroids. This report presents a rare case of Behçet's disease mimicking vertebral invasion of a mycotic aneurysm.
Adrenal Cortex Hormones ; Aneurysm ; Aneurysm, Infected* ; Anti-Bacterial Agents ; Aortic Aneurysm ; Arteries ; Behcet Syndrome ; Blood Vessels ; Diagnosis ; Humans ; Middle Aged ; Osteomyelitis ; Pathology ; Systemic Vasculitis ; Thrombosis ; Ulcer ; Uveitis ; Veins ; Venous Thrombosis

BACKGROUND/AIMS: Anemia in patients with inflammatory bowel disease significantly affects the quality of life. The aim of this study was to investigate the frequency of and risk factors for anemia and to describe the management of anemia in patients with intestinal Behcet's disease (BD) in actual clinical practice. METHODS: We included 64 patients with intestinal BD who visited the outpatient clinic of a tertiary referral center in June 2011 and had available laboratory data for the subsequent 6 months. RESULTS: Anemia was detected in 26 patients (40.6%). After 6 months, anemia was still present in 14 of these patients (53.8%). The cause of anemia was investigated in eight patients (30.8%), and oral iron supplementation was prescribed to four patients (15.4%). Of these four patients, two (50%) recovered completely within 6 months. Anemia was associated with a high Disease Activity Index for Intestinal Behcet's Disease (DAIBD, p=0.024), erythrocyte sedimentation rate (p=0.003), and C-reactive protein (p=0.049) in univariate analysis. In multivariate analysis, the factor predictive for anemia in patients with intestinal BD was a higher DAIBD (> or =40; odds ratio, 4.08; 95% confidence interval, 1.21 to 13.71; p=0.023). CONCLUSIONS: Although anemia is common in intestinal BD patients, its clinical importance is overlooked in daily practice. Moderate to severe disease activity is predictive of anemia.
Adult ; Anemia/drug therapy/epidemiology/*etiology ; Behcet Syndrome/blood/*complications/pathology ; Blood Sedimentation ; C-Reactive Protein/analysis ; Dietary Supplements ; Disease Management ; Female ; Humans ; Intestinal Diseases/blood/*complications/pathology ; Iron/therapeutic use ; Male ; Middle Aged ; Multivariate Analysis ; Odds Ratio ; Predictive Value of Tests ; Risk Factors ; Severity of Illness Index ; Trace Elements/therapeutic use

No abstract available.
Behcet Syndrome/*pathology ; Female ; Humans ; Interleukins/*blood ; Male ; Receptors, Cell Surface/*blood

Behcet's disease (BD) is a multisystem vascular inflammatory disease. BD can affect blood vessels of nearly all sizes and types. Arterial involvement is a rare but serious condition in the course of BD. Here, we report a case of stenosis at the iliac artery bifurcation which was treated with percutaneous balloon angioplasty and stent implantation in a 37-year-old patient with BD.
Adult ; Angioplasty, Balloon ; Behcet Syndrome ; Blood Vessels ; Constriction, Pathologic* ; Humans ; Iliac Artery ; Stents*

PURPOSE: The serum levels of soluble triggering receptor expressed on myeloid cells-1 (sTREM-1) have recently been shown to be correlated highly with disease activity in patients with intestinal Behcet's disease (BD). However, it remains unclear whether sTREM-1 levels reflect endoscopic activity in intestinal BD. This study aimed to evaluate the correlation of sTREM-1 levels with endoscopic activity in intestinal BD. MATERIALS AND METHODS: A total of 84 patients with intestinal BD were enrolled. Endoscopic activity was compared with sTREM-1 levels as well as other laboratory findings, including erythrocyte sedimentation rate (ESR) and C-reactive protein (CRP). RESULTS: sTREM-1 levels were significantly increased in intestinal BD patients compared with controls (37.98+/-27.09 pg/mL vs. 16.65+/-7.76 pg/mL, p=0.002), however, there was no difference between endoscopically quiescent and active diseases (43.53+/-24.95 pg/mL vs. 42.22+/-32.68 pg/mL, p=0.819). Moreover, serum sTREM-1 levels did not differ in terms of number, shape, depth, size, margin, or type of ulcer in patients with intestinal BD. However, mean ESR and CRP levels in patients with active disease were significantly higher than those in patients with quiescent disease (p=0.001, p<0.001, respectively). In addition, endoscopic activity scores for intestinal BD were correlated significantly with both CRP levels (gamma=0.329) and ESR (gamma=0.298), but not with sTREM-1 levels (gamma=0.166). CONCLUSION: Unlike CRP levels and ESR, serum sTREM-1 levels were not correlated with endoscopic activity in patients with intestinal BD.
Adult ; Behcet Syndrome/*blood/*pathology ; Biological Markers/blood ; Blood Sedimentation ; C-Reactive Protein/metabolism ; Female ; Humans ; Intestinal Diseases/*blood/*pathology ; Male ; Membrane Glycoproteins/*blood ; Receptors, Immunologic/*blood

Interleukin (IL)-33 is an important mediator of innate immunity. Behcet's disease (BD) is an autoinflammatory disorder characterized by hyperactivity of the innate immune response. We measured serum levels of IL-33 and its receptor soluble ST2 (sST2) in patients with BD to investigate their association with disease activity. Serum levels of both IL-33 and sST2 were higher in patients with BD compared with those in normal controls (IL-33: 594.48+/-175.04 pg/mL in BD and 224.23+/-56.64 pg/mL in normal controls [P=0.048], sST2: 99.01+/-15.92 pg/mL in BD and 23.56+/-3.25 pg/mL in normal controls [P<0.001]). IL-33 and sST2 expression in skin tissue, as shown by immunohistochemistry, was higher in patients with BD compared with that in the normal controls. Serum sST2 level correlated significantly with the BD currently active form (BDCAF), Iranian BD dynamic activity measure (IBDDAM), erythrocyte sedimentation rate and C-reactive protein. Multiple linear regression showed that serum sST2 was an independent factor associated with IBBDAM (regression coefficient, 0.374; P=0.004), and BDCAF (regression coefficient, 0.236; P=0.047). These results demonstrate that IL-33 and sST2 are highly expressed in patients with BD and that serum sST2 is an independent factor associated with IBDDAM and BDCAF, suggesting a potential role for sST2 as a surrogate marker of disease activity in patients with BD.
Adult ; Behcet Syndrome/blood/*pathology ; Blood Sedimentation ; C-Reactive Protein/analysis ; Female ; Humans ; Immunohistochemistry ; Interleukins/*blood/metabolism ; Male ; Middle Aged ; Receptors, Cell Surface/*blood/metabolism ; Severity of Illness Index ; Skin/metabolism/pathology

PURPOSE: We aimed to determine the prevalence of anti-cyclic citrullinated peptide (anti-CCP) antibodies in a large group of Korean patients with Behcet's disease (BD), with and without joint involvement, and to compare these findings with the prevalences of anti-CCP antibodies in patients with rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). MATERIALS AND METHODS: We tested 189 patients with BD, 105 with RA, and 36 with SLE for anti-CCP antibodies and IgM rheumatoid factor in serum. We reviewed the medical records of patients with BD to investigate their personal and clinical characteristics as well as their laboratory test results. RESULTS: Anti-CCP antibodies were detected in seven of the 189 BD patients (3.7%), at a mean titer of 30.6+/-44.4 U/mL, in 86 of the 105 RA patients (81.9%) with a mean titer of 198.8+/-205.7 U/mL, and in nine of the 36 SLE patients (25%) with a mean titer of 180.4+/-113.9 U/mL. One of the seven anti-CCP-positive BD patients fulfilled the diagnostic criteria for both BD and RA. Five of the seven anti-CCP-positive BD patients (71.4%) had polyarticular joint involvement, and the other two patients (28.6%) had oligoarticular involvement. CONCLUSION: We determined the prevalence of anti-CCP antibodies in a large group of Korean BD patients with and without joint involvement. Negative anti-CCP test in patients with BD may help to differentiate BD from RA and SLE, all of which present with similar clinical features.
Adolescent ; Adult ; Aged ; Antibodies/*blood ; Arthritis, Rheumatoid/blood/immunology ; Behcet Syndrome/*blood/immunology ; Child ; Female ; Humans ; Lupus Erythematosus, Systemic/blood/immunology ; Male ; Middle Aged ; Peptides, Cyclic/*immunology ; Young Adult

No abstract available.
Adult ; Behcet Syndrome/complications/*diagnosis ; Colon/blood supply/pathology/surgery ; Colonoscopy ; Gastrointestinal Hemorrhage/*diagnosis/etiology/surgery ; Humans ; Ileum/blood supply/surgery ; Male ; Rectum/blood supply/surgery

Endovascular repair of inflammatory aortic aneurysms has been reported as an alternative to open surgical treatment. In selective cases, adjunctive bypass surgery may be required to provide an adequate landing zone. We report a case of endovascular repair of an inflammatory aortic aneurysm in a patient with Behcet's disease using a carotid-carotid bypass graft to provide an adequate landing zone. A 45-yr-old man with a voice change was referred to our hospital with the diagnosis of saccular aneurysm of the distal aortic arch resulting from vasculitis. Computed tomography showed a thoracic aortic aneurysm with thrombosis. Right to left carotid-carotid bypass grafting was performed. After 8 days, the patient underwent an endovascular stent graft placement distal to the origin of the innominate artery. The patient was discharged with medication and without postoperative complications after 5 days. Hybrid endovascular treatment may be suitable a complementary modality for repairing inflammatory aortic aneurysms.
Aortic Aneurysm, Thoracic/complications/radiography/*surgery ; Behcet Syndrome/*complications/surgery ; Blood Vessel Prosthesis Implantation/*methods ; Carotid Arteries/physiopathology/*surgery ; Coronary Artery Bypass ; Endovascular Procedures ; Humans ; Male ; Middle Aged ; Stents ; Thrombosis/complications ; Tomography Scanners, X-Ray Computed ; Vasculitis/complications

High-mobility group box 1 (HMGB1) protein has been demonstrated to play an important role in chronic inflammatory diseases including rheumatoid arthritis, and systemic lupus erythematosus. This study investigated the association between extracellular HMGB1 expression and disease activity, and clinical features of Behcet's disease (BD). Extracellular HMGB1 expression in the sera of 42 BD patients was measured and was compared to that of 22 age- and sex-matched healthy controls. HMGB1 expression was significantly increased in BD patients compared to healthy controls (78.70 +/- 20.22 vs 10.79 +/- 1.90 ng/mL, P = 0.002). In addition, HMGB1 expression was significantly elevated in BD patients with intestinal involvement compared to those without (179.61 +/- 67.95 vs 61.89 +/- 19.81 ng/mL, P = 0.04). No significant association was observed between HMGB1 concentration and other clinical manifestations, or disease activity. It is suggested that extracellular HMGB1 may play an important role in the pathogenesis of BD.
Adult ; Aged ; Behcet Syndrome/genetics/*metabolism/pathology ; Extracellular Space/metabolism ; Female ; HMGB1 Protein/genetics/*metabolism ; Humans ; Inflammation ; Intestinal Diseases/blood/genetics ; Male ; Middle Aged ; Young Adult