Cases reported "Behcet Syndrome"

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1/47. Retinal tears associated with panuveitis and Behcet's disease.

    To report retinal tears formation in 3 eyes of 2 patients with active panuveitis and Behcet's disease. We describe 2 patients that were diagnosed and treated for Behcet's disease with active panuveitis. Retinal tears developed while the inflammation was active. The patients were treated with topical, oral steroids, and cyclosporine therapy for bilateral panuveitis. One patient presented with a retinal tear located at the periphery of the active retinal lesion. The other had multiple tears associated with active retinal lesions in both eyes. argon laser photocoagulation was performed in both patients as soon as the tears were detected. Ocular inflammation was controlled with this therapy, and only a few mild flare-ups occurred. The patients have been followed up for 8 and 16 months, respectively. During this period no new retinal tears have developed. Although retinal tear formation is rarely associated with Behcet's panuveitis, the clinician should be aware of this as a possible complication. When structural changes are present in the vitreous, detailed ophthalmoscopy is indicated to assess for retinal tears. If a tear is detected in a patient with panuveitis and Behcet's disease, laser photocoagulation therapy should be performed immediately to prevent retinal detachment.
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2/47. Neutrophil and endothelial cell activation in the vasa vasorum in vasculo-Behcet disease.

    AIM: The aim of this study was to analyse the immunopathological mechanisms of vasculo-Behcet disease, which were also compared to cases of Takayasu's arteritis and inflammatory aneurysm to evaluate differences in inflammatory mechanisms. METHOD AND RESULTS: We reviewed six cases of vasculo-Behcet disease, four of Takayasu's arteritis and seven inflammatory aneurysms which underwent surgical repair. Immunohistochemical studies were performed on paraffin-embedded tissue using a labelled streptavidin-biotin method, as was in-situ hybridization for Epstein-Barr virus. Microscopically, neutrophils and lymphocytes accumulated around the vasa vasorum. neutrophils were prominent as compared to Takayasu's arteritis and inflammatory aneurysm. Elastic fibres were not severely destroyed. endothelial cells (ECs) of most vasa vasorum expressed HLA-DR. The number of vasa vasorum around which inflammatory infiltrating cells were observed in vasculo-Behcet disease was significantly greater than in inflammatory aneurysms and Takayasu's arteritis (P < 0.001). The cytokines IL-1alpha, TNF-beta and IFN-gamma were expressed in neutrophils and lymphocytes which were distributed around vasa vasorum, as well as neutrophils adherent to HLA-DR positive ECs. CONCLUSION: Our results suggest that vasculo-Behcet disease should be classified as a neutrophilic vasculitis targeting the vasa vasorum. aneurysm formation may be related to degeneration of arterial wall caused by inflammation of the vasa vasorum.
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3/47. Macular ischaemia in Behcet's disease.

    PURPOSE: To report macular ischaemia and visual loss in patients with panuveitis due to Behcet's disease. methods: We describe macular ischaemia, a rare finding, in 3 eyes of 3 patients with panuveitis who were diagnosed and treated as having Behcet's disease. The patients underwent fundus fluorescein angiography (FFA) using a digital imaging system and were treated with topical and oral steroids and cyclosporine in 2 cases, and with added azathioprine in the third case. RESULTS: The 3 eyes showed macular ischaemia associated with peripheral retinal vasculitis on FFA and control of inflammation was achieved in all cases. After an average of 2 years follow-up, visual acuity and macular ischaemia improved in 2 eyes, while no recovery was seen in the third. CONCLUSION: The presence of macular ischaemia on FFA in Behcet's disease is a predictor of poor visual outcome. Macular ischaemia may show partial recovery with the treatment of the disease.
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4/47. Behcet's disease: dental and oral soft tissue complications.

    Behcet's disease is a chronic, multisystem disorder. It has 3 primary components: recurrent inflammations of the eye, ulcerations of the oral mucous membranes, and ulcerations of the genitalia. diagnosis of Behcet's disease relies mainly on history taking and clinical manifestations. This article describes the oral soft tissue and dental complications and the prosthetic problems encountered in a young patient. If dentists encounter patients with chronic ocular inflammations and recurrent oral mucous membrane ulcers, the index of suspicion should increase.
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5/47. Efficacy of interferon alfa-2a in severe and refractory uveitis associated with Behcet's disease.

    PURPOSE: To evaluate the efficacy of interferon alfa-2a (IFN alfa) in severe uveitis associated with Behcet's disease, that is refractory to steroids and conventional immunosuppressive agents. patients AND methods: patients with Behcet's disease (according to the International Study Group criteria), who relapsed despite steroids and immunosuppressive agents, were included in this retrospective study. Ophthalmological examination, laser flare photometry, and fluorescein angiography associated with laboratory tests were performed at regular intervals. IFN alfa (3 millions units thrice a week) was injected subcutaneously. RESULTS: Eight patients (sex ratio: 1) were included between May 1995 and January 1999. The mean age was 29.1 years (14-54 years) and the disease was present between 11 and 167 months before the administration of IFN alfa. IFN alfa was efficient in all cases with a mean follow-up of 22 months (10-55 months). steroids were tapered from a mean dosage of 47 mg/d to a mean dosage of 8.5 mg/d. Ocular inflammation was controlled and visual acuity improved in all cases. Treatment was generally well tolerated despite a constant but transient flu-like syndrome. IFN alfa was tapered in three patients and stopped in one case without any relapse after five months. CONCLUSIONS: Within the limitations of this retrospective study, low-dose IFN alfa seems to be well tolerated, promising in the management of refractory forms of uveitis due to Behcet's disease, and effective in allowing a reduction of steroid dosage. A prospective controlled study is necessary to confirm these preliminary results.
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6/47. Atypical Behcet's syndrome in a patient with myelodysplastic syndrome.

    We report the case of a 67-year-old man with myelodysplastic syndrome (MDS), who presented with fever, painless penile and groin ulcers, as well as oral and esophageal ulcerations, all of which were exquisitely responsive to corticosteroids. Some cases of Behcet's syndrome and MDS have been reported in association with trisomy 8 and HLA B51, with the pathology varying from vasculitis to acute neutrophilic inflammation. Our patient with orogenital ulcers had neither trisomy 8 nor HLA B51. Also lacking were other features typical of Behcet's syndrome, such as uveitis, vasculitis, and central nervous system abnormalities. However, his response to corticosteroids on the initial presentation and subsequent episodes was dramatic. Esophageal ulcerations have not been described in Behcet's syndrome. In this respect, our patient was unique.
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7/47. Coincidence of recurrent arthritis and Behcet's disease following anterior cruciate ligament reconstruction.

    Chronic recurrent inflammation with joint effusion following anterior cruciate ligament (ACL) reconstruction is common. We report an unusual case of a patient also suffering from undiagnosed Behcet's disease. The patient had chronic recurrent joint effusions of the knee 7 years after traumatic rupture of the ACL and autologous tendon reconstruction using a hamstring tendon autograft. Arthroscopic treatment of the notch impingement and therapy of the accompanying rheumatic disease relieved the patient of his ailment. On the one hand, this case report shows the necessity of including chronic rheumatoid joint disease in the differential diagnosis of a persistent impairment of joint homeostasis following ACL reconstruction, and on the other hand it shows the coincidence of Behcet's disease and recurrent joint inflammation in a patient following ACL surgery.
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8/47. Ileal perforation in diffuse intestinal Behcet disease: report of a case.

    Intestinal ulcers in Behcet disease tend to cause perforation, and postoperative recurrence is common with a high mortality rate. The optimal therapeutic strategy has yet to be elucidated, particularly in cases of diffuse intestinal involvement. We herein present a case of diffuse intestinal Behcet disease with ileal perforation. A 57-year-old Japanese woman was referred to our institution with complaints of intractable oral ulcers and abdominal pain. The patient underwent an emergency laparotomy for perforated peritonitis in spite of the intravenous administration of prednisolone (1.5 mg/kg) under total parenteral nutrition. Macroscopically, an inflamed ileum measuring 1.6 m in length was resected, including a 1-cm perforated ulceration. Innumerable small and deep ulcers were also observed, consisting of nonspecific inflammation. The patient has been free from any recurrence of intestinal ulcers while being treated with prednisolone, colchicine, and a low-residue diet for 1.5 years.
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9/47. Current therapy for Behcet's disease.

    Classified among the vasculitides, the clinical spectrum of Behcet's disease (BD) ranges from a mild mucocutaneous disease to a life-threatening systemic vasculitis, characterized by remissions and recurrences. The major morbidity is recurrent eye inflammation that may lead to blindness, but severe central nervous system, gastrointestinal, or vascular involvement may occur and might be fatal. In contradistinction to most other vasculitides, the venous system is commonly affected in BD. The treatment of BD is usually symptomatic and palliative. This includes topical steroids for orogenital ulcers, nonsteroidal antiinflammatory agents for joint involvement, and colchicine as prophylaxis against disease flares (although evidence that colchicine prevents recurrences of oral and genital ulcers is restricted to female patients). Immunosuppressives and cytotoxic agents are used for more severe involvement, and thalidomide and interferon have attracted attention in recent years.
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10/47. Long-term opioid treatment in Behcet's syndrome with intractable abdominal pain--a case report.

    Opioids are the most potent and effective analgesics available for treating acute and chronic cancer pain, but its usefulness in treating non-cancer chronic pain is still controversial. We report a 23-year-old male suffering from Behcet's disease with persistent excruciating abdominal pain. Oral non-steroid anti-inflammation analgesics and milder opioids (codeine and tramadol) failed to relieve the pain. His excruciating abdominal pain resulting from ulcerative viscera aggravated with repeated gastrointestinal tract surgeries. Intravenous morphine given through patient-controlled analgesia (PCA) could effectively reduce his pain with minimal side effects. No sign of physical or psychological dependence was observed during the period of opiate administration, and no withdrawal phenomenon was found as the dosage was tapered. During the total treatment course of 213 days, the daily dose of morphine once surged up to 259.2 mg. The long-term opioid treatment and its possible effects are, herein, discussed.
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