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

BACKGROUND: Neuropsychiatric systemic lupus erythematosus (NPSLE) shows some similarities to neuroBehcet's disease (NBD) in that both conditions have some analogous clinical features and they are both pathologically associated cerebral vasculopathy. This study compared the clinical manifestations, brain MRI findings and prognosis of NPSLE and NBD patients. METHODS: Forty three patients with NPSLE (n = 25) or NBD (n = 18), who were monitored at a single center, were enrolled in this study. We retrospectively analyzed the clinical and brain MRI data. The neuropsychiatric manifestations were classified in both groups according to the new American College of Rheumatology nomenclature for NPSLE. RESULTS: The diffuse symptoms that included mood disorders, psychosis, confusion, cognitive dysfunctions, generalized seizures and headaches other than migraine or cluster headaches were more commonly observed in the NPSLE patients, while the frequency of focal diseases such as cranial neuropathy tended to be higher in the NBD patients. The brain MRI revealed that the NBD patients had more abnormalities in the brain stem than did the NPSLE patients. Most of the patients improved, at least partially, after being treated with glucocorticoid and/or immune suppressants. However, the disease course differed significantly between the two groups. There were more episodic cases in the NPSLE group of patients, while there were more remittent cases in the NBD group of patients. CONCLUSION: NPSLE had a tendency to cause diffuse neuropsychiatric manifestations, and it has a different predilection of brain lesions compared with NBD. The NBD patients showed a poorer outcome than did the NPSLE patients, suggesting that different therapeutic strategies for the two diseases need to be considered.
Adult ; Behcet Syndrome/complications/*diagnosis/pathology ; Brain/*pathology ; Female ; Humans ; Lupus Erythematosus, Systemic/complications/diagnosis/pathology ; Lupus Vasculitis, Central Nervous System/complications/*diagnosis/pathology ; *Magnetic Resonance Imaging ; Male ; Prognosis ; Retrospective Studies ; Risk Factors

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

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

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

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

Behcet's disease has been recognized as a systemic vasculitis characterized by the involvement of multiple organs such as orogenital ulcers, eye lesions including uveitis and optic neuritis, and skin lesions including folliculitis and erythema nodosum. Vascular involvement occurs occasionally and is classified into thrombosis and aneurysm. However, massive gastrointestinal bleeding from arterial aneurysm is a rare manifestation of intestinal Behcet's disease. Recently, we experienced a case of intestinal Behcet's disease presenting with massive gastrointestinal bleeding due to aneurysmal rupture of ileo-colic artery. A 30-year-old male with Behcet's disease was admitted because of massive gastrointestinal bleeding. A large ileo-cecal ulcer was revealed as a bleeding focus on colonoscopic examination. Celiac angiography showed aneurysm and stenosis of ileo-colic artery. After the failure of hemostasis with arterial embolization, ileocecectomy was performed. After the resection hematochezia was completely stopped.
Adult ; Aneurysm, Ruptured/complications/*diagnosis ; Behcet Syndrome/complications/*diagnosis ; Cecum/*blood supply/pathology/surgery ; Celiac Artery/radiography ; Colonoscopy ; Gastrointestinal Hemorrhage/*diagnosis/etiology ; Humans ; Ileum/*blood supply/pathology/surgery ; Male ; Tomography, X-Ray Computed

BACKGROUND/AIMS: Behcet's disease (BD) is a systemic disorder associated with a characteristic vasculitis that can involve both veins and arteries of all sizes. Endothelial activation or injury is a characteristic feature of BD. Endothelial dysfunction is widely regarded as being the initial lesion in the development of atherosclerosis. The carotid artery intima-media thickness (IMT) is a widely accepted marker of subclinical atherosclerosis. We aimed to determine the carotid IMT in BD patients with using high-resolution B-mode Doppler ultrasonography. METHODS: We studied 40 patients (24 males, mean age: 39.1+/-8.5 years) who were diagnosed by the international diagnostic criteria of Behcet's disease and 20 healthy controls (13 males, mean age: 40.2+/-5.1 years), and the two groups were matched by age and gender. No subject in either group had a history of atherosclerosis or its complications. The clinical data, including the age of onset, the duration of disease, a history of medication, the activity score and the laboratory data were analyzed. RESULTS: The carotid IMT in the BD group was significantly higher than that in the control group (0.71+/-0.22 mm vs. 0.59+/-0.09 mm, respectively, p<0.01). Cardiac and major vessel involvements were not identified in the BD group. However, minor vascular involvements were documented in 2 patients with deep vein thrombosis, in 4 patients with superficial thrombophlebitis and in 2 patients with pseudoaneurysm. The carotid IMT in the patients with posterior uveitis or retinal vasculitis was higher than that of the patients without these findings (0.85+/-0.21 mm vs. 0.64+/-0.10 mm, respectively, p=0.007), but there was no difference of the IMT according to minor vascular involvement. CONCLUSIONS: Despite that there was no significant cardiovascular involvement in the BD patients, the carotid IMT was significantly higher in the BD patients as compared with the healthy controls.
Adult ; Behcet Syndrome/complications/diagnosis/*pathology ; Carotid Arteries/*pathology/ultrasonography ; Case-Control Studies ; Endothelium, Vascular/pathology ; Female ; Humans ; Male ; Prognosis ; Prospective Studies ; Risk Factors ; Tunica Intima/*pathology/ultrasonography ; Tunica Media/*pathology/ultrasonography ; Ultrasonography, Doppler ; Vasculitis/pathology

Arterial and cardiac involvement of Behcet's disease is a rare but life threatening complication. The rupture of an arterial aneurysm might result in sudden death. We report a 54-year-old man with an established diagnosis of Behcet's disease who presented with multiple cardiovascular complications that eventually lead to his death. He presented with extensive venous occlusions, and sequentially developed right ventricular thrombosis with multiple pulmonary thromboembolisms, and a pulmonary artery aneurysm. We report this unusual sequence of cardiovascular complications in a patient with Behcet's disease.
Aneurysm, Dissecting/*etiology ; Behcet Syndrome/*complications ; Cardiovascular Diseases/diagnosis/*etiology/ultrasonography ; Fatal Outcome ; Hemoptysis ; Humans ; Male ; Middle Aged ; Pulmonary Artery/*pathology ; Pulmonary Embolism/*etiology ; Risk Factors ; Rupture/etiology ; Tomography, X-Ray Computed

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