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

No abstract available.
Adult ; Behcet Syndrome/*complications/diagnosis ; Follow-Up Studies ; Humans ; Male ; Optic Disk/*pathology ; Optic Neuritis/diagnosis/*etiology ; Time Factors ; Visual Acuity

PURPOSE: Behcet's disease (BD) theoretically affects all sizes and types of blood vessels and results in multi-organ involvement. However, renal BD has not been fully characterized, though the kidneys are histologically rich in blood vessels. MATERIALS AND METHODS: A total of 2007 patients who fulfilled the diagnostic criteria for BD were enrolled in this study. We reviewed the medical records and test results of the BD patients and used univariate and multivariate logistic regression analyses to determine the clinical significance of renal involvement in BD. RESULTS: Among the 2007 BD patients, we noted hematuria in 412 (20.5%) and proteinuria in 29 (1.4%). Univariate analysis showed that the BD patients with hematuria were predominantly female and older, had higher erythrocyte sedimentation rates (ESRs), and more frequently presented with genital ulcerations. BD patients with proteinuria had higher ESR levels compared to BD patients without proteinuria. In the multivariate analysis, age, sex, and ESR were found to be significantly associated with hematuria in BD patients, whereas only ESR was associated with proteinuria in BD patients. We also found that IgA nephropathy was the most common pathologic diagnosis in 12 renal BD patients who underwent renal biopsies. CONCLUSION: We suggest that routine urinalysis and serum renal function tests be performed for the early detection of renal BD, especially in older female BD patients with recurrent hematuria, high ESR levels, and frequent genital ulcers, as well as in BD patients with proteinuria and high ESR levels.
Adolescent ; Adult ; Age Factors ; Aged ; Aged, 80 and over ; Behcet Syndrome/*complications/epidemiology/*metabolism ; Biopsy ; Female ; Glomerulonephritis, IGA/complications/diagnosis ; Hematuria/complications/diagnosis ; Humans ; Kidney/*pathology ; Kidney Diseases/*diagnosis ; Logistic Models ; Male ; Middle Aged ; Multivariate Analysis ; Proteinuria/complications/diagnosis ; Republic of Korea ; Young Adult

We report an unusual case of unilateral frosted branch angiitis associated with Behcet's disease, including a review of previously reported cases. A 39-year-old male with history of recurrent oral and genital ulcers presented with visual loss in his left eye. Fundus findings demonstrated occlusive retinal vasculitis resembling acute frosted branch angiitis. Laboratory examinations including viral markers revealed no abnormal findings except positive HLA-B51. The patient was treated with systemic steroid and cyclosporine. Six months after presentation, new oral ulcers and pseudofolliculitis appeared, and he was diagnosed with Behcet's disease following rheumatology consultation. During follow-up, there was no change in visual acuity of hand movement, and disc neovascularization developed even after complete panretinal photocoagulation. Ocular manifestations of Behcet's disease can present as unilateral frosted branch angiitis, and may consecutively involve in both eyes. Early immunosuppressive treatment is recommended.
Adult ; Behcet Syndrome/*complications/diagnosis ; Diagnosis, Differential ; Fluorescein Angiography ; Fundus Oculi ; Humans ; Magnetic Resonance Imaging ; Male ; Retinal Artery/*pathology ; Retinal Vasculitis/diagnosis/*etiology

Behcet's disease (BD) is a multisystem inflammatory disorder that presents as recurrent oral and genital ulcers in conjunction with other dermatological and ocular manifestations. The prevalence of BD is higher in Middle and East Asia than in Western countries. Intestinal BD is a specific subtype of BD, characterized by intestinal ulcers and associated gastrointestinal symptoms. Similar to inflammatory bowel disease, intestinal BD exhibits a fluctuating disease course with repeated episodes of relapse and remission that necessitate adequate maintenance therapy after achievement of clinical remission. Medical treatment of intestinal BD is largely empirical since well-controlled studies have been difficult to perform due to the heterogeneity and rarity of the disease. To date, 5-aminosalicylic acid, systemic corticosteroids, and immunosuppressants have been used anecdotally to treat intestinal BD. The clinical course of intestinal BD shows considerable variability, and the exact point at which more potent agents such as immunosuppressants should be used has not yet been elucidated. Given the difficulty in predicting which patients will experience complicated disease courses and the fact that these drugs are related with certain risk resulting from immunosuppression, proper identification of prognostic factors in intestinal BD may allow physicians to implement tailored medical therapy and individualized patient monitoring based on risk stratification. In this review, the impact of baseline characteristics on the long-term course of intestinal BD, prognostic factors during various medical therapies, and outcome predictors related to surgery will be discussed.
Adrenal Cortex Hormones/adverse effects/therapeutic use ; Adult ; Age Factors ; Behcet Syndrome/*diagnosis/pathology/therapy ; Female ; Humans ; Immunosuppressive Agents/adverse effects/therapeutic use ; Immunotherapy ; Intestinal Diseases/*diagnosis/pathology/therapy ; Male ; Prognosis ; Sex Factors

OBJECTIVETo investigate the clinical features, diagnosis methods, therapeutic principles of intestinal Behcet's disease.

METHODSking Union Medical College Hospital from August 1998 to April 2010 were retrospectively analyzed.

RESULTSThe clinical courses of patients with intestinal Behcet's disease were from 26 days to 33 years, and the average duration was 6.32 +/- 1.01 years. The appearance of extra-gastrointestinal symptoms was significantly earlier than that of gastrointestinal symptoms (7.35 +/- 1.39 years vs. 3.24 +/- 0.82 years, P<0.05). The predominant gastrointestinal manifestations were right lower quadrant pain (95.56%) and hematochezia or melena (40.00%). Misdiagnosis occurred in 17 cases. In patients without systemic medicine therapy before surgery, the incidence of postoperative infection of incision site and abdominal cavity was significantly higher than that in those undergoing systemic medicine therapy (80.00% vs. 0%, P<0.05).

CONCLUSIONSBecause of the diversity of gastrointestinal manifestations, intestinal Behcet's disease is easily misdiagnosed. The systemic medical therapy before surgery could decrease the incidence of infection of incision and abdominal cavity.

Adolescent ; Adult ; Aged ; Behcet Syndrome ; diagnosis ; pathology ; surgery ; Child ; Female ; Gastrointestinal Diseases ; diagnosis ; pathology ; surgery ; Humans ; Intestinal Diseases ; diagnosis ; pathology ; surgery ; Male ; Middle Aged ; 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

Behcet's disease is a multisystemic inflammatory disease characterized with recurrent oral ulcer, genital ulcer, and multiple organ involvement. Aplastic anemia is one of the rarest complications of Behcet's disease. There were only several reports about Behcet's disease associated myelodysplatic syndrome worldwide. Moreover, aplastic anemia in intestinal Behcet's disease was rarely reported. Here, we present a case of aplastic anemia with trisomy 8 and trisomy 9 in intestinal Behcet's disease and a review of the literatures. To the authors' knowledge, this is the first case ever reported in Korea.
Adult ; Anemia, Aplastic/complications/*diagnosis ; Behcet Syndrome/complications/*diagnosis/genetics ; Bone Marrow/pathology ; Chromosomes, Human, Pair 8 ; Chromosomes, Human, Pair 9 ; Female ; Humans ; Intestinal Diseases/complications/*diagnosis/genetics ; Karyotyping ; Tomography, X-Ray Computed ; *Trisomy

PURPOSE: Behcet's disease (BD) is a chronic, relapsing, multi-system vasculitis of unknown aetiology with complicated and diversified clinical features predominantly involving oral and genital ulcers, and ocular and cutaneous lesions. The clinical features of this disease have been described to be different according to geographical areas and gender. We investigated the specific clinical features of BD patients in Northeastern China. MATERIALS AND METHODS: 116 patients involved in this study fulfilled the classification criteria of the International Study Group for BD. The clinical manifestations and results of laboratory tests of BD were recorded in each patient. RESULTS: The onset was typically between 20-39 years with a slight female predominance. Oral ulcers were the most common manifestation, followed by skin lesions, positive pathergy reaction/genital ulcers, and ocular lesions. Vascular lesion and epididymitis were rare in patients with BD. The frequency of erythema nodosum-like lesion and articular involvement were significantly higher in females, while gastrointestinal involvement was significantly higher in males. The results of laboratory tests showed that the human leukocyte antigen (HLA)-B*51 alleles were positive in 30.9% of patients and the immunological abnormities were present in some patients. CONCLUSION: The clinical features of BD showed geographical and gender difference. Genetic and immune factors might participate in aetiopathogenesis of BD.
Behcet Syndrome/*diagnosis/epidemiology/pathology ; China ; Female ; Geography ; Humans ; Male

Behcet's disease (BD) has been recognized as multi-systemic chronic vasculitic disorder of recurrent inflammation, characterized by the involvement of multiple organs and resulting in orogenital ulcers, uveitis, and skin lesions. Involvement of the central nervous system, vessels, and intestines in BD often leads to a poor prognosis. Digestive manifestations in BD have been reported in up to 1-60% of cases, although the rate varies in different countries. The most frequent extra-oral sites of gastrointestinal involvement are the ileocecal region and the colon. Gastric or esophageal involvement is reported to be very rare. Moreover, there have been no reports on the simultaneous involvement of the esophagus, stomach, ileum, and colon. Here, we present a 55-year-old Korean man with intestinal BD and multiple ileal and colonic ulcerations complicated by perforation, gastric ulcer with bleeding followed by perforation, and esophageal ulcers with bleeding.
Behcet Syndrome/complications/*diagnosis/pathology ; Cecal Diseases/complications/pathology ; Diagnosis, Differential ; Endoscopy, Digestive System ; Gastrointestinal Diseases/complications/*diagnosis ; Gastrointestinal Hemorrhage ; Humans ; Intestinal Perforation/*diagnosis/etiology/pathology ; Male ; Middle Aged ; Peptic Ulcer Perforation/pathology ; Stomach Ulcer/complications/pathology