Behcet disease

  • 文章类型: Case Reports
    甲状腺中的淀粉样蛋白沉积是常见的表现,然而淀粉样甲状腺肿仍然相对罕见。这种情况与其他甲状腺肿类型和恶性肿瘤的正确区分是至关重要的。虽然淀粉样蛋白广泛侵入甲状腺,患者通常甲状腺功能正常,和许多不同的演示可能发生。我们报告了一例42岁的男性患者,该患者因Behcet病被诊断为继发性淀粉样变性。他的临床表现为甲状腺功能亢进和系统性淀粉样变性并发慢性肾脏病,这是巴勒斯坦报告的第一个这样一个实体的案例。
    Amyloid deposition in the thyroid gland is a common presentation, yet amyloid goiter remains relatively rare. Proper differentiation of this condition from other goiter types and malignancies is essential. Although amyloid extensively invades the thyroid gland, patients are usually euthyroid, and many different presentations may occur. We report a case of a 42-year-old male patient who was diagnosed with secondary amyloidosis due to Behcet\'s disease. He presented with clinical manifestations of hyperthyroidism and systemic amyloidosis complicated by chronic kidney disease, which is the first case of such an entity to be reported in Palestine.
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  • 文章类型: Journal Article
    背景:作为可变血管血管炎的Behcet病(BD)的主要特征是眼部受累,生殖器和口腔口疮,和结节性红斑.然而,主要器官受累,包括胃肠道受累,神经系统,血管受累是严重并发症之一。骨坏死是BD患者的罕见并发症。我们的目标是报告最大的一系列患有骨坏死的BD患者。
    方法:我们回顾性回顾了伊朗Behcet疾病登记处的所有患者,并报道了骨坏死患者。患者用药和临床特征,症状,骨坏死的细节也将介绍。此外,以前报告的病例也将得到审查。
    结果:诊断为BD并登记的患者为7,800,31例。18例患者发生ON,发生率为0.22%。疾病进展过程中最常见的受累是口腔口疮,100%的患者出现,其次是眼部受累85.7%,皮肤受累71.4%。血管,眼,骨坏死BD患者的神经系统受累率明显高于其他BD患者。对于葡萄膜炎急性发作的治疗,深静脉血栓形成,严重的胃肠道受累,动脉受累,神经系统的参与,和关节受累高剂量的糖皮质激素表示。
    结论:ON往往表现为BD患者的多灶性受累,因此,在一个关节中诊断为ON后,应调查其他可能的ON部位。
    BACKGROUND: Behcet disease (BD) as a variable vessel vasculitis is mainly characterized by ocular involvement, genital and oral aphthosis, and erythema nodosum. However, major organ involvements including gastrointestinal involvement, nervous system, and vascular involvement are among the severe complications. Osteonecrosis is a rare complication of patients with BD. We aim to report the largest series of BD patients suffering from osteonecrosis.
    METHODS: We have retrospectively reviewed all patients in Iran Behcet\'s Disease Registry and reported those with osteonecrosis. Patients\' medication and clinical features, symptoms, and details of osteonecrosis will also be presented. Furthermore, previously reported cases will also be reviewed.
    RESULTS: Seven thousand eight hundred thirty-one patients were diagnosed with BD and registered. 18 patients developed ON with an incidence of 0.22%. The most common involvement during the disease progression was oral aphthosis which appeared in 100% of patients followed by ocular involvement in 85.7% and skin involvement in 71.4%. Vascular, ocular, and nervous system involvements are significantly higher in BD patients with osteonecrosis than the other BD patients. For the management of acute episode of uveitis, deep vein thrombosis, severe gastrointestinal involvement, arterial involvement, nervous system Involvement, and joint involvement high dose of glucocorticoids is indicated.
    CONCLUSIONS: ON tends to appear as a multifocal involvement in BD patients, hence, after diagnosis of ON in one joint other possible sites of ON should be investigated.
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  • 文章类型: Systematic Review
    慢性炎症代表了炎症性风湿性疾病(IRD)患者心血管(CV)风险升高的基石,包括血管炎.与普通人群相比,这些患者的标准化死亡率更高,过早死亡的原因是早期动脉粥样硬化事件。因此,IRD患者需要根据这种CV疾病(CVD)负担进行适当的CV风险评估和管理。在常规护理中仍然缺乏对CV风险的充分控制,但早期诊断无症状和亚临床CVD对改善这些患者的长期预后至关重要.增加的动脉僵硬度可能在炎症和增加的心血管风险之间提供病理生理联系。现在有几种非侵入性方法可以在临床上评估动脉硬度,包括脉搏波速度评估。动脉僵硬度对心血管事件的独立预测价值已在一般和选定人群中得到证实。建议调整年龄和血压的参考值。因此,动脉僵硬度是心血管危险分层的一个有趣的生物标志物.本系统综述总结了PWV测量在血管炎中可以提供的附加价值,重点放在不同的临床阶段和CV风险预防上。本系统评价注册编号:ProsperoCRD42021259603。
    Chronic inflammation represents the cornerstone of the raised cardiovascular (CV) risk in patients with inflammatory rheumatic diseases (IRD), including vasculitis. Standardized mortality ratios in these patients are higher as compared to the general population, and the excess of premature mortality is due to early atherosclerotic events. Thus, IRD patients need appropriate CV risk assessment and management according to this CV disease (CVD) burden. Adequate control of CV risk is still lacking in usual care, but early diagnosis of silent and subclinical CVD is crucial to improve the long-term prognosis of these patients. Increased arterial stiffness may provide a pathophysiological link between inflammation and increased cardiovascular risk. Several noninvasive methods are now available to estimate artery stiffness in the clinical setting, including pulse wave velocity assessment. The independent predictive value of arterial stiffness for cardiovascular events has been demonstrated in general as well as in selected populations, and reference values adjusted for age and blood pressure have been suggested. Thus, arterial stiffness is an interesting biomarker for cardiovascular risk stratification. This systematic review summarizes the additional value that PWV measurement can provide in the setting of vasculitis, with a focus in the different clinical stages and CV risk prevention. This systematic review is registered with registration number: Prospero CRD42021259603.
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  • 文章类型: Case Reports
    Behçet\'s disease (BD) is vasculitis affecting vessels of variable sizes characterized with recurrent oral and/or genital aphthous ulcers accompanied by cutaneous, ocular, articular, gastrointestinal, and/or central nervous system inflammatory lesions. The disease is characterized by recurrent attacks and remissions of different durations, which is one of the reasons why the diagnosis is, in most cases, made several years after the onset of first symptoms. We present a 24-year old male, with South Eastern European heritage, with relapsing bilateral optic neuritis as a first symptom of the disease, followed by aseptic meningitis 2 years, and vascular manifestation 3 years after onset, which finally led to the diagnosis of Behçet\'s disease. Vascular symptoms were thromboembolism of the right leg and aneurism of the right popliteal artery that due to the size required surgical treatment. The patient was treated with glucocorticoids, azathioprine and anti-tumor necrosis factor-alpha therapy, that proved to be the best treatment options for all manifestations of the disease. Based on our literature review, optic neuritis is a known and rare clinical feature of BD. To our knowledge, there are only several literature reports in which optic neuritis is the initial symptom of BD. Our case report and literature review emphasize the importance of recognizing optic neuritis without inflammatory eye disease as a possible presenting symptom of BD and accentuate detailed medical history review at each patient\'s visit.
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  • 文章类型: Case Reports
    Behçet病(BD)是一种慢性多系统疾病,其系统性表现为血管炎。在BD的过程中很少诊断出心脏受累,尤其是左心室心内血栓,并且通常与预后不良有关。心内血栓的原因尚不清楚。导致内皮细胞损伤和高凝的特定促炎途径有助于心脏中血栓形成是合理的。已知的血栓因子如亚甲基四氢叶酸还原酶基因突变,因子V莱顿突变,蛋白质S和C,抗凝血酶III,活化蛋白C抗性,抗磷脂抗体可能有助于BD心内血栓的形成。我们报告了一例24岁的男性BD患者,伴有左心室血栓。经胸超声心动图可以描述和监测这种罕见的心脏表现。高剂量皮质类固醇和硫唑嘌呤的组合在10天内成功溶解了心内血栓,而没有抗凝。
    Behçet disease (BD) is a chronic multisystem disorder with vasculitis underlying its systemic manifestations. Cardiac involvement and particularly left ventricular intracardiac thrombus are rarely diagnosed in the course of BD and are often associated with poor prognosis. The causes of intracardiac thrombi are unknown. It is plausible that specific proinflammatory pathways resulting in the endothelial cell injury and hypercoagulation contribute to the formation of thrombotic masses in the heart. Known thrombophilic factors such as methylenetetrahydrofolate reductase gene mutations, factor V Leiden mutation, proteins S and C, antithrombin III, activated protein C resistance, and antiphospholipid antibodies may contribute to the formation of intracardiac thrombi in BD. We report a case of a 24-year-old male patient with BD presented with left ventricular thrombus. Transthoracic echocardiography allowed to describe and monitor such a rare cardiac manifestation of the disease. A combination of high-dose corticosteroid and azathioprine successfully dissolved intracardiac thrombus within ten days without anticoagulation.
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