Cases reported "Behcet Syndrome"

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1/10. Peripheral vascular disease in Behcet's syndrome.

    We describe a patient with Behcet's syndrome who had peripheral vascular disease involving the left subclavian artery for which angioplasty with stent placement was performed and reangioplasty done for in-stent restenosis. She presented with recurrence one year after stent placement; angiography revealed diffuse disease of the axillary and radial arteries with mild to moderate restenosis at the site of stent placement.
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2/10. Cryopreserved aortic homograft replacement in 3 patients with noninfectious inflammatory vascular disease.

    OBJECTIVE: Although mechanical prosthetic heart valves are most commonly used for aortic valve replacement in patients with aortic regurgitation due to noninfectious inflammatory vascular disease, postoperative perivalvular leakage and/or detachment of the prosthetic valve occurs due to the fragility of the aortic annulus. Aortic root replacement with cryopreserved homografts is reported to be useful in such patients. methods: Three patients having aortic regurgitation associated with severe long standing noninfectious inflammatory vascular disease-2 patients with Takayasu's arteritis and 1 patient with Behcet disease--had the aortic root replacement by a cryopreserved aortic homograft valve and conduit. RESULTS: All surgery was successful and the postoperative course uneventful. echocardiography showed neither aortic regurgitation nor graft detachment at 6-39 months after operation. CONCLUSIONS: Homograft valve and conduit replacement is appropriate in patients with aortic regurgitation associated with noninfectious inflammatory vascular disease, with mid-term results favorable.
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ranking = 1.4
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3/10. Renal Behcet's disease: a cumulative analysis.

    OBJECTIVE: To analyze cumulated data about renal involvement in Behcet's disease (BD) and to report on 6 patients with BD and renal problems. methods: We found reports of 159 patients (including our patients) with BD and specific renal disease (amyloidosis 69, glomerulonephritis [GN] 51, renal vascular disease 35, and interstitial nephritis 4) in our survey. RESULTS: The frequency of renal problems among BD patients has been reported to vary between 0% to 55%. Male gender is a risk factor for all types of renal BD. nephrotic syndrome was present in 83% of patients with amyloidosis, and renal failure was common at the time of diagnosis. The mean interval between the initial manifestation of BD and diagnosis of amyloidosis was shorter in men than in women (P =.02). AA-type amyloid fibrils were shown in all cases studied. Vascular involvement was common in the patients with amyloidosis (60%). The renal findings in GN show a wide spectrum, from asymptomatic hematuria and/or proteinuria to rapidly progressive GN. Several types of glomerular lesions ranging from minor glomerular changes to crescentic glomerulonephritis are observed in BD. The common types of glomerular lesions among the reported cases are crescentic GN, proliferative GN, and immunoglobulin a (IgA) nephritis. Aneurysms may be located throughout the renal artery, from the orifice of the main artery to intrarenal microaneurysms. Another type of renal disease (amyloidosis or GN) and other major vascular involvement were present in all cases with renal vein thrombosis. hypertension is common among patients with renal artery aneurysm or stenosis. Microscopic vascular disease was described in 4 patients. CONCLUSIONS: Based on data in the literature, we suggest that renal involvement in BD is more frequent than has been recognized, although it is most often mild in nature. amyloidosis is one of the prognostic factors affecting survival. patients with vascular involvement carry high risk for amyloidosis, and administration of colchicine to these patients may be beneficial. More evidence is needed to accept interstitial nephritis as a manifestation of BD. In spite of some difficulties, hemodialysis and renal transplantation are safe treatment options in BD-related uremia.
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ranking = 0.4
keywords = vascular disease
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4/10. Vascular manifestations of Behcet's disease. Eighteen cases among 140 patients.

    OBJECTIVES: To describe the features, prognosis, and treatment of vascular involvement in Behcet's disease (BD). patients: Among 140 patients with BD seen at the Hotel-Dieu Hospital in Beirut between 1980 and 2000, 18 (13%) had vascular involvement and were included in this retrospective study. All these patients fulfilled International Study Group criteria for BD. RESULTS: Men with BD were more likely to have vascular involvement (13/77, 17%) than women (5/63, 8%) (P = 0.12) and were younger at diagnosis of vascular disease (32 /- 7 vs. 36 /- 7.5 years; P < 0.01). Many patients had vascular disease at more than one site: 17 had thrombophlebitis, 10 had arterial thromboses, and one had an aneurysm. thrombophlebitis was more common in men (82% vs. 18%; P < 0.03) and arterial occlusion in women (70% vs. 30%; P > 0.05). Caval thrombosis and arterial occlusions were the most serious complications. Combined treatment with glucocorticoids, anticoagulants, and immunosuppressants was effective in superior vena cava syndrome and extracranial arterial occlusion. CONCLUSION: Vascular manifestations of BD are common in lebanon, particularly venous lesions. Aneurysms are seen less often than arterial occlusions. Medical treatment may be sufficient in superior vena cava syndrome and arterial occlusion.
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5/10. Cerebral venous sinus thrombosis in Behcet's disease: a case report.

    Behcet's disease ( BD ) is a systemic inflammatory vascular disease with variable clinical manifestations and numerous clinical masquerades. The venous involvement may affect veins of different size with tendency for thrombosis. BD is the commonest cause of cerebral venous thrombosis in some Middle Eastern countries. However, in thailand, this clinical entity has been rarely described as a cause of cerebral venous sinus thrombosis. A 38 year-old man presented with recurrent attacks of transient ischemic attack (TIA) for 9 months. Two months after the TIA, he developed right hemiparesis and persistent diffuse headache. He had a 10 year history of recurrent oral and genital ulcers. physical examination revealed bilateral uveitis, right hemiparesis, bilateral sixth cranial nerve paresis and bilateral papilloedema. Pathergy skin test showed positive result. neuroimaging demonstrated empty delta sign on CT-scan and thrombosis of the posterior part of superior saggital sinus, transverse sinus, straight sinus and internal jugular vein on magnetic resonance venography (MRV). cerebrospinal fluid (CSF) analysis revealed increased intracranial pressure and aseptic CSF profiles. prednisolone, chlorambucil and anticoagulant had been prescribed. The clinical course improved gradually.
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6/10. A large left ventricular pseudoaneurysm in Behcet's disease: a case report.

    BACKGROUND: Behcet's disease is a collagen-vascular disease most commonly seen in asia and Mediterranean area. Different organs and systems including cardiovascular system could be involved. Pseudoaneurysm is the most common form of arterial involvement in Behcet's disease; however, cardiac pseudoaneurysm is rare. CASE PRESENTATION: A rare case of 13 years old boy with a 4-year history of Behcet's disease with development of a huge left ventricular pseudoaneurysm is reported who had been admitted because of cough, chills, fever, and chest pain. Findings obtained on echocardiography, magnetic resonance imaging, chest computed tomography and coronary angiography confirmed a left ventricular pseudoaneurysm. There was no complication for next 24 months follow up period after surgical treatment. CONCLUSION: Considering its fatality and nonspecific manifestations, one should consider cardiac pseudoaneurysms as a potential risk in any patient with Behcet's disease.
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ranking = 0.2
keywords = vascular disease
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7/10. MR brain scanning in patients with vasculitis: differentiation from multiple sclerosis.

    We performed MR (magnetic resonance) brain imaging on 24 patients with a systemic vasculitis. MRI proved to be a sensitive method for detecting brain lesions (clinically silent or manifest) in these patients. The most frequent abnormalities were periventricular lesions seen in 12 cases. Such changes are not specific for vascular disease, and are often seen in multiple sclerosis. However, additional changes were commonly seen which suggested the correct diagnosis.
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8/10. Vascular surgery in Behcet's syndrome: a case report.

    A case of Behcet's syndrome with right femoral artery thrombosis surgically treated by femoro-popliteal tea and lumbar sympathectomy is described. The validity of tea even in this rare vascular disease is evidenced. After having examined the reasons leading to this surgical procedure, and good results in long-term follow-up are underlined.
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9/10. Clinical study on death in Behcet's disease.

    We were able to identify 7 patients who died of Behcet's disease, among 2,200 patients registered at the Behcet's disease Specialty Clinic of Severance Hospital from November 1983 to October 1992. Six were male and one female. Age of death was 31 to 55 years with the mean age of 39.1. The age of onset was 24 to 54 years with the mean age of 32.6. The mean duration of illness was 6.5 years. The most frequent initial symptom was oral ulcer, with the most common type being the incomplete type in Shimizu's classification. Positive pathergy tests were found in all the 5 patients in whom the test was performed out of 7 patients. Various modalities of treatments such as oral steroid, colchicine, azathioprine, colectomy, and pacemaker insertion were attempted without outstanding therapeutic effects. The causes of death were gastrointestinal bleeding, bowel perforation, superior and inferior vena cava syndrome, aortic regurgitation, cerebrovascular disease, sepsis, and lung abscess.
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ranking = 0.2
keywords = vascular disease
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10/10. Bilateral loss of vision and macular ischemia related to Behcet disease.

    PURPOSE: To report a 61-year-old man with sudden onset of visual loss in both eyes related to bilateral macular ischemia. methods: The patient underwent comprehensive ophthalmic examination including slit-lamp and fundus examination, fluorescein angiography, and visual field testing as well as biologic screening. RESULTS: Bilateral macular ischemia associated with peripheral retinal vasculitis was confirmed by angiography. We diagnosed Behcet disease by association of ocular, oral, and cutaneous involvement according to the criteria of the international study group for Behcet disease. The patient was treated with corticosteroids. CONCLUSION: In Behcet disease, sudden onset of bilateral vision loss may be associated with bilateral retinal vascular disease and macular ischemia. Prompt diagnosis and treatment with systemic corticosteroids may be beneficial.
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