Behcet disease

  • 文章类型: Case Reports
    急性心肌梗死(AMI)是青年患者的重要问题,因为它对健康和社会生活的影响。年轻人的ST抬高型心肌梗死(STEMI)的机制和病程可能与老年人不同。白塞病(BD)是一种病因不明的多系统自身免疫性疾病。心脏受累很少,然而据报道,它影响了6%的患者,17%的病例表现为第一表现。我们介绍了一个33岁的男性重度吸烟者,病史阴性,出现急性下壁心肌梗死.他的冠状动脉造影显示右冠状动脉近端有巨大的血栓形成。他接受了原发性冠状动脉介入治疗和药物洗脱支架植入治疗,随后在48h内因急性支架内血栓形成而介入并植入另外两个药物洗脱支架。风湿病学评估显示有4次不同的口腔溃疡发作和1次生殖器溃疡发作史。他的检查显示人白细胞抗原(HLA)等位基因(B51)阳性,与BD密切相关。青年动脉血栓形成导致的AMI可归因于与BD早期表现有关的高凝状态。增加年轻人对AMI的认识及其在BD中的表现对于降低发病率和死亡率是必要的。糖皮质激素和秋水仙碱可改善BD的心脏表现。
    Acute myocardial infarction (AMI) in young patients is an important issue because of its impact on health and social life. The mechanisms and disease courses of ST-elevation myocardial infarction (STEMI) in young individuals may differ from those in the elderly. Behcet disease (BD) is a multisystem autoimmune disorder of unknown etiology. Cardiac involvement is rare, yet it was reported to affect 6% of patients, with 17% of the cases presenting as the first manifestation. We present the case of a 33-year-old male heavy smoker with negative medical history, who presented with acute inferior myocardial infarction. His coronary angiography showed huge thrombosis in proximal right coronary artery. He was treated with primary coronary intervention and implantation of drug-eluting stent, with subsequent intervention and implantation of two more drug-eluting stents due to acute stent thrombosis within 48 h. Rheumatologic assessment revealed the history of four different attacks of oral ulcers and one attack of genital ulcer. His workup showed positive human leukocyte antigen (HLA) allele (B51) which is strongly associated with BD. AMI in young adults due to arterial thrombosis can be attributed to hypercoagulable state related to early manifestation of BD. Increased knowledge of AMI in young adults and its presentation in BD is necessary to reduce morbidity and mortality. Corticosteroids and colchicine may improve cardiac manifestations in BD.
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  • 文章类型: Case Reports
    背景:Behçet病(BD)是一种多系统复发性炎症性疾病,最初被描述为伴有葡萄膜炎的口腔和生殖器溃疡的三联征(Behcet,1937年[1])。动脉受累是BD患者死亡的最常见原因。动脉瘤在动脉病变中很常见,并影响各种动脉,但主要是腹主动脉.7%-29%的患者会遇到血管病变,严重影响疾病的进程。由于Behçet病引起的颅外颈动脉动脉瘤极为罕见(Bouarhroum等人。(2006)[2])。
    方法:这里,我们向我们的门诊诊所介绍了一名19岁的男子,该男子因压力效应而出现了蹄形。
    由于计算机血管造影的发现,他接受了两次手术。在头臂动脉分叉处展开100*8覆盖的支架。然后展开与先前支架重叠2cm的40*13.5FLUENCY支架。
    结论:建议对这种罕见疾病的血管内途径进行进一步研究。
    BACKGROUND: Behçet disease (BD) is a multisystemic recurrent inflammatory disorder that was originally described as a triad of oral and genital ulcerations with uveitis (Behcet, 1937 [1]). Arterial involvement is the most common cause of mortality in patients with BD. Aneurysms are common among the arterial lesions and affect various arteries, but mostly the abdominal aorta. Vascular lesions are encountered in 7 %-29 % of patients, gravely affecting the course of the disease. Extracranial carotid aneurysms due to Behçet\'s disease are extremely rare (Bouarhroum et al. (2006) [2]).
    METHODS: Herein, we present a 19 year old man presented with hoarsness due to pressure effect to our outpatient clinic.
    UNASSIGNED: Due to findings in the computed angiography, he underwent surgery twice.A 100*8 COVERA-covered stent was deployed at the bifurcation of the brachiocephalic artery. Then a 40*13.5 FLUENCY stent with a 2 cm overlap from the previous stent was deployed.
    CONCLUSIONS: Further investigations regarding endovascular approach for this rare disease is recommended.
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  • 文章类型: Journal Article
    口炎(UVAL)是一种主要未知的疾病,具有广泛而复杂的鉴别诊断。
    为了提供对UVAL的主要特征的描述,确定最合适的诊断过程并描述当前的治疗方法。
    我们设计了一个回顾展,使用我们机构的妇科ER数据库进行描述性队列研究。纳入标准:年龄在10至20岁之间的女性患者,怀疑在CHUV的妇科ER诊断为UVAL。数据提取:流行病学特征,临床表现,实验室测试,已建立的诊断,治疗,和溃疡的结果。
    包括15例患者进行分析;平均年龄:15岁;溃疡发作时,60%的患者为处女病;所有患者均至少有一种流感样症状伴有外阴病变;最有效的血清学检查是针对EBV,仅有一名患者出现急性疾病;出于诊断目的,进行了两次活检,两种均不确定的组织病理学分析。扑热息痛,和利多卡因凝胶;93%的病例出现消退迹象;平均随访时间为10天。Sadoghi等人的诊断算法:15例中有10例通过该算法回顾性诊断为UVAL;一半被诊断为UVAL,另一半在妇科急诊就诊时被诊断为“来历不明的溃疡”。
    我们强烈推荐Sadoghi等人开发的诊断和治疗算法。作为指导临床推理的宝贵工具,因此,改善急性外阴溃疡的管理。
    UNASSIGNED: Ulcus Vulvae Acutum Lipschütz (UVAL) is a largely unknown disease with a broad and complex differential diagnosis.
    UNASSIGNED: To provide a description of the main characteristics of UVAL, determine the most appropriate diagnostic process and describe the current therapeutic approach.
    UNASSIGNED: We designed a retrospective, descriptive cohort study using the gynecological-ER database of our institution. Inclusion criteria: female patients aged between 10 and 20 years old with suspicion of a UVAL diagnosis at CHUV\'s gynecological ER. Data extraction: epidemiological characteristics, clinical presentation, laboratory tests, established diagnostics, treatment, and ulcer outcomes.
    UNASSIGNED: 15 patients were included for the analysis; average age: 15 years old; 60% of patients were virgo at the time of ulcer onset; all patients had at least one flu-like symptom concomitant with the vulvar lesion; the most-performed serology was for EBV and acute disease was present in only one patient; for diagnostic purposes two biopsies were performed with both inconclusive histopathology analysis; the main prescribed treatments were: oral NSAIDs, Paracetamol, and Lidocaine gel; 93% of cases presented signs of regression; the average follow-up time was 10 days. The diagnostic algorithm of Sadoghi et al: 10 out of 15 cases were retrospectively diagnosed with UVAL by the algorithm; half were diagnosed with UVAL, and the other half received a diagnosis of \"ulcers of unknown origin\" at the time of the gynecological ER visit.
    UNASSIGNED: We highly recommend the diagnostic and therapeutic algorithms developed by Sadoghi et al. as valuable tools to guide clinical reasoning and, consequently, improve acute vulvar ulcers management.
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  • 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: Case Reports
    Behçet病(BD)是一种多系统复发性自身免疫性血管疾病。它的临床特征是复发性口腔溃疡,生殖器溃疡,眼睛,和皮肤表现。BD病例中神经系统症状的发展很少见,并且在最初诊断后几年发生。我们描述了一名39岁的沙特男性的罕见病例,他表现出孤立的神经系统表现是BD的第一个迹象。病人中风复发,缺血性和出血性,在开发典型BD功能之前的11个月内。彻底的调查排除了他的神经系统疾病的其他潜在病因。影像学显示多个脑干病变与实质神经BD(NBD)相容。该患者的HLA-B51阳性,这是一种与BD相关的遗传标记,但做了一个阴性的体形测试.糖皮质激素和英夫利昔单抗治疗可改善症状。NBD的诊断需要全面的临床,成像,和实验室评估以排除其他可能的原因。该病例表明需要在具有无法解释的神经系统表现的年轻患者的鉴别诊断中包括NBD,特别是如果它们之后是BD特征的发作。用皮质类固醇和生物制剂治疗可以取得良好的结果。NBD可以表现为孤立的神经症状,强调需要高度怀疑和多学科方法来准确诊断和有效管理。
    Behçet disease (BD) is a multisystemic relapsing autoimmune vascular disorder. It is clinically characterized by recurrent oral ulcers, genital ulcers, eye, and skin manifestations. Development of neurological symptoms in BD cases is rare and occurs several years after the initial diagnosis. We describe a rare case of a 39-year-old Saudi male who presented with isolated neurological manifestations as the first sign of BD. The patient had recurrent strokes, both ischemic and hemorrhagic, over an 11-month period before developing typical BD features. A thorough investigation excluded other potential etiologies of his neurological disorders. Imaging showed multiple brainstem lesions compatible with parenchymal neuro-BD (NBD). The patient was positive for HLA-B51, a genetic marker linked to BD, but had a negative pathergy test. Treatment with corticosteroids and infliximab resulted in symptom improvement. The diagnosis of NBD requires a comprehensive clinical, imaging, and laboratory assessment to rule out other possible causes. This case demonstrates the need to include NBD in the differential diagnosis of young patients with unexplained neurological manifestations, especially if they are followed by an onset of BD features. Treatment with corticosteroids and biologic agents can achieve favorable outcomes. NBD can present with isolated neurological symptoms, emphasizing the need for a high level of suspicion and a multidisciplinary approach for accurate diagnosis and effective management.
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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: Case Reports
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  • 文章类型: Case Reports
    该病例报告强调了将Behcet病作为口腔生殖器溃疡和葡萄膜炎的诊断,尽管由于漏报,其患病率在尼泊尔未知。此外,需要相关专业之间的患者护理合作。
    This case report highlights considering Behcet\'s disease as a diagnosis in orogenital ulcers and uveitis, although its prevalence is unknown in Nepal due to underreporting. Also, collaboration for patient care among relevant specialties is required.
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  • 文章类型: Case Reports
    结节性多动脉炎(PAN)是一种可能影响多器官系统血管的多系统疾病。它具有临床变体,包括单器官疾病和仅皮肤PAN。据我们所知,这是一个独特的病例报告,描述了在Behçet病(BD)患者中外阴和视网膜的PAN共存。我们报告了一例31岁的黎巴嫩妇女,患有疼痛的生殖器溃疡和与关节痛相关的多个口腔口疮,灯闪烁,视力模糊,和光敏性。有很好的定义,颊粘膜和牙龈粘膜上穿孔的糜烂;特别是,多次打孔,边界不明显的深部溃疡和黑色焦痂,包括三分之二的大阴唇和小阴唇,保留阴蒂,伴有双侧腹股沟淋巴结肿大。眼底扩张检查显示一些棉绒斑点和视网膜内出血。荧光素眼底血管造影显示双眼多发性小动脉梗塞累及黄斑,右眼更是如此。由于中型皮下动脉受累,外阴活检与PAN一致,并显示其壁有嗜中性粒细胞浸润。弹性层染色显示中等大小的皮下动脉受累。在排除传染病病因后,她接受静脉脉冲甲基强的松龙1000毫克治疗三天,然后口服泼尼松龙50毫克,缓慢锥度,口服秋水仙碱0.5毫克,每日两次,和阿达木单抗40mg,每两周一次,以阻止眼部损伤和生殖器切割的进展。溃疡明显改善,没有新的皮肤或全身表现。该病例报告强调了在Behçet病病例中考虑PAN样病变的重要性。我们强调皮肤PAN的添加是BD的皮肤表现之一。
    Polyarteritis nodosa (PAN) is a multisystem disease that may affect the vessels of multiple organ systems. It has clinical variants including single-organ disease and cutaneous-only PAN. To our knowledge, this is a unique case report describing the coexistence of PAN of the vulva and retina in a Behçet\'s disease (BD) patient.  We report a case of a 31-year-old Lebanese woman with painful genital ulcers and multiple oral aphthae associated with arthralgia, light flashes, blurry vision, and photosensitivity. There were well-defined, punched-out erosions over the buccal and gingival mucosa; specifically, multiple punched-out, deep ulcers with unremarkable borders and black eschar involving two-thirds of both labia majora and minora sparing the clitoris with bilateral inguinal lymphadenopathy. Dilated fundus examination showed a few cotton wool spots and intraretinal hemorrhage. Fundus fluorescein angiography showed multiple arteriolar infarctions involving the macula in both eyes, more so in the right eye. Vulvar biopsy was consistent with PAN due to the involvement of a medium-sized subcutaneous artery and showed neutrophilic infiltration of its wall. Stain for elastic lamina showed medium-sized subcutaneous artery involvement. After ruling out infectious aetiologies, she was managed by intravenous pulse methylprednisolone 1,000 mg for three days, followed by oral prednisolone 50 mg with a slow taper, oral colchicine 0.5 mg twice daily, and adalimumab 40 mg once every two weeks to stop the progression of the ocular insult and genital mutilation. There was significant improvement of the ulcer with no new cutaneous or systemic manifestations. This case report highlights the importance of considering PAN-like lesions in cases of Behçet\'s disease. We emphasized the addition of cutaneous PAN as one of the cutaneous manifestations of BD.
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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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