Behçet's disease

Beh ç et 病
  • 文章类型: Journal Article
    目标:患有Behçet病的人,和许多患有慢性病的人一样,经常面对抑郁症,焦虑,生活质量差和性问题。在这项研究中,它的目的是评估抑郁症,焦虑,和白塞病患者的性功能障碍。
    方法:总共100名参与者,50名患者(29名女性)和50名健康志愿者(28名女性),参与研究。贝克抑郁量表(BDI),贝克焦虑量表(BAI),女性性功能指数(FSFI)并对参与者进行国际勃起功能指数(IIEF)。
    结果:抑郁和性功能障碍与Behçet病显著相关。在我们的研究中,所有患有白塞氏病的女性参与者都有性功能问题。勃起功能障碍在Behcet患者中更为常见。结果还表明,抑郁与高潮功能之间存在显着关系(p=0.004),性欲(p=0.028),性满意度(p=0.023),和总体满意度(p=0.028)。白塞氏病患者(10.54±6.45)与健康组(7.36±6.13)的抑郁评分差异有统计学意义(p=0.009)。发现全身受累的患者和粘膜皮肤受累的患者在BDI和BAI评分方面相似(p>0.05)。
    结论:Behçet病被发现是抑郁和性功能障碍的危险因素。
    People with Behçet\'s Disease, as many individuals with chronic diseases, often face depression, anxiety, poor quality of life and sexual problems. In this study, it was aimed to evaluate depression, anxiety, and sexual dysfuntions in people with Behcet\'s Disease.
    A total of 100 participants, 50 patients (29 female) and 50 healthy volunteers (28 female), participated in the study. Beck Depression Inventory (BDI), Beck Anxiety Inventory (BAI), Female Sexual Function Index (FSFI), and International Index of Erectile Function (IIEF) were administered to the participants.
    Depression and sexual dysfunctions were significantly related with Behçet\'s Disease. In our study, all female participants with Behçet\'s Disease had problems in sexual functions. Erectile dysfunction was more frequent in participants with Behcet\'s. The results also showed that there is a significant relationship between depression and orgasmic function (p=0.004), sexual desire (p=0.028), sexual satisfaction (p=0.023), and general satisfaction (p=0.028). There was a significant difference between people with Behçet\'s Disease (10.54±6.45) and healthy group (7.36 ±6.13) in depression scores (p=0.009). Patients with systemic involvement and those with mucocutaneous involvement were found to be similar in terms of BDI and BAI scores (p>0.05).
    Behçet\'s Disease was found to be a risk factor for depression and sexual dysfunctions.
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  • 文章类型: Case Reports
    Behcet病是一种多系统炎症性疾病,其血管受累非常罕见。动脉瘤性动脉受累是这种疾病的严重形式,鉴于其严重性,它构成了治疗挑战,和频繁的继发性并发症。股动脉瘤(PFAA)极为罕见。系统的免疫抑制药物治疗和血管内或外科技术的彻底性可以降低复发率。我们报告了一个年轻人的病例,其中股动脉的假动脉瘤揭示了Behcet病。他接受了使用线圈栓塞的血管内治疗;3个月后控制良好。
    Behcet\'s disease is a multisystem inflammatory disorder, whose vascular involvement is very rare. Aneurysmal arterial involvement is the severe form of the disease, it constitutes a therapeutic challenge given its severity, and frequent secondary complications. Profunda femoral artery aneurysms (PFAAs) are extremely rare. The systematic immunosuppressive drug treatment and endovascular or surgical technique thoroughness can reduce relapse rate. We report the case of a young man in whom a false aneurysm of the profunda femoral artery revealed Behcet\'s disease. He underwent an endovascular treatment by embolization using coils; with good control after 3 months.
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  • 文章类型: Journal Article
    背景:迟发性Behçet病(LBD)的病程未知。本研究旨在确定LBD的特点和系统参与。
    方法:在本回顾性研究中,横截面,和比较研究,从4100例诊断为Behçet病(BD)的患者中选择700份患者档案,并分为三组:LBD,成人BD(ABD),青少年BD(JBD)。LBD和JBD的年龄限制分别为42和16。LBD患者在人口统计学方面与ABD和JBD患者进行比较,系统性参与,和疾病持续时间。
    结果:BD患者的LBD率为4.7%(183/4,100)。LBD从最初症状发生到达到BD标准的年龄的时间比ABD或JBD更长。除了生殖器溃疡,LBD的受累频率与ABD和JBD相似。LBD(12%)的家族史频率显着低于ABD(15.9%)或JBD(18.2%)。从最初症状到口疮的时间,生殖器溃疡,LBD的眼部受累时间长于ABD或JBD。此外,与ABD相比,LBD在更长的持续时间后观察到结节性红斑。
    结论:考虑到LBD的受累时间要晚得多,除了生殖器溃疡,受累频率没有差异,LBD患者应与ABD和JBD患者一样密切随访。
    BACKGROUND: The course of late-onset Behçet\'s disease (LBD) is unknown. This study aimed to determine the characteristics and systemic involvement of LBD.
    METHODS: In this retrospective, cross-sectional, and comparative study, 700 patient files from 4100 patients diagnosed with Behçet\'s disease (BD) were selected and divided into three groups: LBD, adult BD (ABD), and juvenile BD (JBD). The age limits for LBD and JBD were determined to be 42 and 16, respectively. LBD patients were compared to ABD and JBD patients in terms of demographics, systemic involvement, and disease duration.
    RESULTS: The LBD rate among BD patients was 4.7% (183/4,100). The time from initial symptom occurrence to the age at which the BD criteria were met was longer in LBD than in ABD or JBD. Except for genital ulcers, the frequencies of involvement in LBD were similar to those in ABD and JBD. The frequency of family history was significantly lower in LBD (12%) than in ABD (15.9%) or JBD (18.2%). The time from the initial symptom to oral aphthae, genital ulcers, and eye involvement was longer in LBD than in ABD or JBD. Furthermore, erythema nodosum was observed after a longer duration in LBD than in ABD.
    CONCLUSIONS: Considering that involvement occurs much later in LBD and there are no differences in the frequencies of involvement except for genital ulcers, LBD patients should be followed up as closely as ABD and JBD patients.
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  • 文章类型: Case Reports
    Behçet病是一种罕见的以口腔溃疡为特征的多系统血管炎,生殖器溃疡,皮肤和眼部病变。Neuro-Behçet综合征是一种情况,其中患有Behçet疾病的个体会出现无法归因于其他神经系统疾病的神经系统症状。我们介绍了一例罕见的神经-Behçet综合征,伴有急性核间眼肌麻痹和神经功能恶化,先前有复发性口腔溃疡的病史,并伴有动脉样特征。痤疮样丘疹脓疱皮疹,视网膜出血,和不伴生殖器溃疡的复发性附睾炎。患者用环磷酰胺改善。此病例强调了在没有生殖器溃疡的情况下诊断和管理神经Behçet综合征的重要性。
    Behçet\'s disease is a rare multisystemic vasculitis characterized by oral ulcers, genital ulcers, and skin and ocular lesions. Neuro-Behçet\'s syndrome is a condition in which individuals with Behçet\'s disease experience neurological symptoms that cannot be attributed to other neurological diseases. We present a rare case of neuro-Behçet\'s syndrome with acute internuclear ophthalmoplegia and deteriorating neurological function with a prior history of recurrent oral ulcers with pathergy-like features, acneiform papulopustular rash, retinal hemorrhages, and recurrent epididymitis without genital ulcers. Patient improved with cyclophosphamide. This case underscores the importance of diagnosing and managing neuro-Behçet\'s syndrome in the absence of genital ulcers.
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  • 文章类型: Journal Article
    Behçet疾病(BD)是一个复杂的医学难题,迷人的研究人员以其神秘的发病机理。这种复杂的疾病,以复发性口腔和生殖器病变为特征,眼睛刺激,和皮肤损伤,对治疗研究提出了重大障碍。这篇综述探讨了microRNAs(miRNAs)与BD的复杂相互作用,强调他们在疾病的病理生理学中的关键参与。miRNA,被公认为在不同生物过程中的监管影响,在自身免疫性疾病的分子机制中占有举足轻重的地位,例如BD。探索从研究miRNA生物通路和功能开始,建立对他们监管机制的基本理解。转向控制BD的分子景观,这篇综述强调了miRNA介导的对Notch等关键信号通路的影响,核因子κ-活化B细胞的轻链增强子(NF-κB),和蛋白激酶B(AKT)/哺乳动物雷帕霉素靶蛋白(mTOR),提供对复杂病理生理机制的见解。剖析免疫学景观揭示了miRNA对BD的深刻影响,阐明免疫反应的复杂调节,并为疾病的病因和进展提供新的观点。除了分子的复杂性,这篇综述探讨了miRNA在BD中的临床意义,强调它们作为诊断和预后指标的潜力。讨论扩展到基于miRNA的治疗干预的有希望的领域,强调它们在缓解症状和改变疾病进展方面的潜力。这次全面审查,作为研究人员的宝贵资源,临床医生,和利益相关者,旨在破译BD复杂的分子挂毯,探索miRNAs的治疗潜力。
    Behçet\'s Disease (BD) is an intricate medical puzzle, captivating researchers with its enigmatic pathogenesis. This complex ailment, distinguished by recurrent mouth and genital lesions, eye irritation, and skin injuries, presents a substantial obstacle to therapeutic research. This review explores the complex interaction of microRNAs (miRNAs) with BD, highlighting their crucial involvement in the disease\'s pathophysiology. miRNAs, recognized for regulatory influence in diverse biological processes, hold a pivotal position in the molecular mechanisms of autoimmune diseases, such as BD. The exploration begins with examining miRNA biogenic pathways and functions, establishing a foundational understanding of their regulatory mechanisms. Shifting to the molecular landscape governing BD, the review highlights miRNA-mediated impacts on critical signaling pathways like Notch, nuclear factor kappa-light-chain-enhancer of activated B cells (NF-κB), and protein kinase B (AKT)/mammalian target of rapamycin (mTOR), offering insights into intricate pathophysiological mechanisms. Dissecting the immunological landscape reveals the profound influence of miRNAs on BD, shedding light on the intricate modulation of immune responses and offering novel perspectives on disease etiology and progression. Beyond molecular intricacies, the review explores the clinical relevance of miRNAs in BD, emphasizing their potential as diagnostic and prognostic indicators. The discussion extends to the promising realm of miRNA-based therapeutic interventions, highlighting their potential in alleviating symptoms and altering disease progression. This comprehensive review, serving as a valuable resource for researchers, clinicians, and stakeholders, aims to decipher the intricate molecular tapestry of BD and explore the therapeutic potential of miRNAs.
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  • 文章类型: Journal Article
    白塞病(BD)是一种全身性血管炎,可累及许多不同的器官系统。神经受累(NBD)发生在5.3%至59%的BD患者中。诊断具有挑战性,尤其是在首次出现神经系统表现的情况下,基于一系列临床,实验室,和神经影像学发现。NBD可以通过免疫介导的脑膜脑炎细分为实质NBD,并偏爱脑干,基底神经节,丘脑,颅神经,和脊髓受累,脑静脉血栓形成和颅内动脉受累的实质外NBD。脑磁共振在T2-FLAIR图像上显示高信号强度的水肿不明确区域,基底神经节区或脑干的T1加权图像中的等强度或低信号,可能延伸到间脑结构。可能会注意到肿胀。出血可以看到,如对比增强(血脑屏障破坏)。磁共振静脉造影和计算机断层血管造影可用于诊断实质外NBD。实质形式的治疗基于与口服免疫抑制剂相关的糖皮质激素(硫唑嘌呤,霉酚酸酯或甲氨蝶呤)在温和的形式,和静脉内环磷酰胺或英夫利昔单抗的严重形式。脑血栓形成的治疗包括与口服抗凝治疗相关的类固醇疗程。必须尽早认识到这种情况,才能开始适当的治疗,以改善结果并限制后遗症的风险。复发,或死亡。这篇综述的目的是总结关于BD的各种神经系统表现的全面综述,强调诊断工具,预后,和治疗问题。
    Behcet disease (BD) is a systemic vasculitis which can involve many different organ systems. Neurological involvement (NBD) occurs in 5.3% to 59% of BD patients. The diagnosis is challenging especially in case of inaugural neurological presentation, and is based on a constellation of clinical, laboratory, and neuroimaging findings. NBD can be subdivided into parenchymal NBD through an immune mediated meningoencephalitis with a predilection to the brainstem, basal ganglia, thalamus, cranial nerves, and spinal cord involvement, and extraparenchymal NBD encompassing cerebral veinous thrombosis and intracranial arterial involvement. Brain magnetic resonance shows ill-defined areas of oedema with high signal intensity on T2-FLAIR images, isointense or hypointense in T1-weighted images in the basal ganglia area or in the brainstem, which may extend to the diencephalic structures. Swelling might be noticed. Hemorrhages can be seen, such as contrast enhancement (blood brain barrier disruption). Magnetic resonance venography and computerized tomographic angiography can be used to diagnose extraparenchymal NBD. Treatment of parenchymatous forms is based on glucocorticoids associated with oral immunosuppressants (azathioprine, mycophenolate mofetil or methotrexate) in mild forms, and intravenous cyclophosphamide or infliximab in severe forms. The management of cerebral thrombosis consists of steroids course associated with an oral anticoagulation. An early recognition of this condition is mandatory to initiate adequate therapies in order to improve outcomes and limit the risk of sequelae, relapses, or death. The aim of this review is to summarize a comprehensive review on the various neurological presentations of BD with emphasizes on diagnostic tools, prognosis, and therapeutic issues.
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  • 文章类型: Journal Article
    背景:患有Behçet病(BD)的患者很少表现为类似于多发性硬化(MS)的脑白质病变。由于实质神经BD(pNBD)和MS之间的诊断困难,这可能导致误诊。本研究旨在通过关注临床和实验室特征,阐明BD和MS(BDMS)合并症患者与仅pNBD和MS患者的区别特征。我们还旨在通过将BD+MS患者与pNBD和MS患者进行比较来确定BD+MS患者的独特特征。
    方法:本研究的方法涉及对伊斯坦布尔医学院神经内科患者记录的回顾性分析,伊斯坦布尔大学。研究人群包括诊断为pNBD的患者,MS,BD和MS(BD+MS)共病。我们评估了临床,放射学,和实验室数据,包括疾病发作,年复发率,扩展的残疾状况量表(EDSS)进展,和脑脊液检查。在pNBD之间检查了几个参数,MS,和BD+MS患者组,以发现亚组之间的异同。
    结果:我们的研究包括1,764例患者:172例pNBD,1,574与MS,和18与BD+MS。在BD+MS中,女性占主导地位(72%,p<0.001)和MS(69%,与pNBD(30%)相比,p<0.001)组。BD+MS出现神经系统症状的中位年龄为35.5(IQR:16.8)岁,pNBD为34.6(13.6)年,MS为27.6(13.3)年(BD+MSvs.MS;p=0.3,pNBD与MS,p=0.7)。此外,攻击的数量明显不同,BD+MS患者的发作中位数为3.5(2.0),而MS患者为3.0(3.0),pNBD患者仅为1.0(1.0),提示与pNBD相比,MS和BD+MS组的病程更活跃(p<0.001)。BD+MS的中位年复发率为0.3(0.2),低于MS(p=0.048)中0.4(0.4)的比率,相当于pNBD中0.2(0.3)的比率(p=0.2)。第一次复发的时间与BD+MS和MS相似,但明显短于pNBD患者(p<0.0001)。脑脊液(CSF)分析显示BDMS和MS患者的中性粒细胞和淋巴细胞计数没有显着差异,但pNBD患者的水平升高(p<0.05)。所有组的CSF蛋白水平是一致的(p=0.1和p=0.7)。在所有BD+MS患者中均检测到寡克隆条带,在大多数MS患者(83.6%)中,和一小部分pNBD患者(19.7%),显示BD+MS和pNBD组之间的显著区别(p<0.001)。
    结论:我们的研究强调了在诊断和治疗表现出症状性MS样MRI病变的BD患者中需要一种怀疑的方法。我们的研究结果表明,BD+MS是一种独特的临床实体,保证具体的诊断和治疗方法。我们的发现强调,符合MS诊断标准的MS样病变的BD患者应作为MS和BD合并症而不是pNBD患者进行管理。
    BACKGROUND: Patients with Behçet\'s disease (BD) may rarely manifest with cerebral white matter lesions resembling multiple sclerosis (MS). This may result in misdiagnosis due to diagnostic difficulties between parenchymal neuro-BD (pNBD) and MS. This study aims to elucidate the distinguishing features of patients with comorbid BD and MS (BD+MS) in comparison to those with pNBD and MS alone by focusing on clinical and laboratory features. We also aimed to identify the distinctive characteristics of BD+MS patients by comparing them to patients with pNBD and MS.
    METHODS: The methodology of this study involved a retrospective analysis of patient records followed in the Department of Neurology at the Istanbul Faculty of Medicine, Istanbul University. The study population included patients diagnosed with pNBD, MS, and a comorbid condition of BD and MS (BD+MS). We assessed clinical, radiological, and laboratory data, including disease onset, annual relapse rates, Expanded Disability Status Scale (EDSS) progression, and cerebrospinal fluid examination. Several parameters were examined between the pNBD, MS, and BD+MS patient groups to find similarities and differences between subgroups.
    RESULTS: Our study included 1,764 patients: 172 with pNBD, 1,574 with MS, and 18 with BD+MS. A predominance of females was noted in the BD+MS (72%, p < 0.001) and MS (69 %, p < 0.001) groups compared to pNBD (30 %). The median age at the onset of neurological symptoms was 35.5 (IQR: 16.8) years for BD+MS, 34.6 (13.6) years for pNBD, and 27.6 (13.3) years for MS (BD+MS vs. MS; p = 0.3, pNBD vs. MS, p = 0.7). Additionally, the number of attacks was notably different, with BD+MS patients experiencing a median of 3.5 (2.0) attacks compared to 3.0 (3.0) for MS patients and only 1.0 (1.0) for pNBD patients, suggesting a more active disease course in the MS and BD+MS groups compared to pNBD (p < 0.001). The median annualized relapse rate for BD+MS was 0.3 (0.2), which was lower than the rate of 0.4 (0.4) in MS (p = 0.048) and equivalent to the rate of 0.2 (0.3) in pNBD (p = 0.2). The time to the first relapse was similar to those with BD+MS and MS, but considerably shorter than in individuals with pNBD (p < 0.0001). The cerebrospinal fluid (CSF) analysis showed no significant differences in neutrophil and lymphocyte counts between BD+MS and MS patients but elevated levels in pNBD patients (p < 0.05). CSF protein levels were consistent across all groups (p = 0.1 and p = 0.7). Oligoclonal bands were detected in all patients with BD+MS, in the majority of MS patients (83.6 %), and a small percentage of pNBD patients (19.7 %), showing a notable distinction between the BD+MS and pNBD groups (p < 0.001).
    CONCLUSIONS: Our study underscores the need for a skeptical approach in diagnosing and treating patients with BD who exhibit symptomatic MS-like MRI lesions. Our findings suggest that BD+MS is a distinct clinical entity, warranting specific diagnostic and treatment approaches. Our findings highlight that BD patients with MS-like lesions meeting MS diagnostic criteria should be managed as patients with comorbid MS and BD rather than pNBD.
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  • 文章类型: Journal Article
    Behçet病(BD)是一种影响多个器官系统的复杂自身免疫性疾病。虽然腹主动脉瘤(AAA)在BD的参与是罕见的,它可能会带来严重的后果。在本研究中,我们确定了BD合并AAA患者的诊断性生物标志物。最初使用孟德尔随机化(MR)分析来探索BD和AAA之间的潜在因果关系。Limma包裹,WGCNA,采用PPI和机器学习算法来识别潜在的诊断基因。建立列线图的受试者工作特征曲线(ROC),以确定BD患者AAA的诊断价值。最后,进行免疫细胞浸润分析和单样本基因集富集分析(ssGSEA)。MR分析表明BD与AAA风险之间存在暗示性关联(比值比[OR]:1.0384,95%置信区间[CI]:1.0081-1.0696,p=0.0126)。使用整合的生物信息学分析鉴定了三个hub基因(CD247,CD2和CCR7),随后用于构建列线图(曲线下面积[AUC]:0.982,95%CI:0.944-1.000)。最后,免疫细胞浸润实验显示,失调的免疫细胞与三个hub基因呈正相关。我们的MR分析显示,BD患者对AAA的易感性更高。我们使用了系统的方法来鉴定三个潜在的枢纽基因(CD247,CD2和CCR7),并开发了列线图来协助BD患者中AAA的诊断。此外,免疫细胞浸润分析表明免疫细胞比例失调。
    Behçet\'s disease (BD) is a complex autoimmune disorder impacting several organ systems. Although the involvement of abdominal aortic aneurysm (AAA) in BD is rare, it can be associated with severe consequences. In the present study, we identified diagnostic biomarkers in patients with BD having AAA. Mendelian randomization (MR) analysis was initially used to explore the potential causal association between BD and AAA. The Limma package, WGCNA, PPI and machine learning algorithms were employed to identify potential diagnostic genes. A receiver operating characteristic curve (ROC) for the nomogram was constructed to ascertain the diagnostic value of AAA in patients with BD. Finally, immune cell infiltration analyses and single-sample gene set enrichment analysis (ssGSEA) were conducted. The MR analysis indicated a suggestive association between BD and the risk of AAA (odds ratio [OR]: 1.0384, 95% confidence interval [CI]: 1.0081-1.0696, p = 0.0126). Three hub genes (CD247, CD2 and CCR7) were identified using the integrated bioinformatics analyses, which were subsequently utilised to construct a nomogram (area under the curve [AUC]: 0.982, 95% CI: 0.944-1.000). Finally, the immune cell infiltration assay revealed that dysregulation immune cells were positively correlated with the three hub genes. Our MR analyses revealed a higher susceptibility of patients with BD to AAA. We used a systematic approach to identify three potential hub genes (CD247, CD2 and CCR7) and developed a nomogram to assist in the diagnosis of AAA among patients with BD. In addition, immune cell infiltration analysis indicated the dysregulation in immune cell proportions.
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  • 文章类型: Journal Article
    Behçet病(BD)是一种病因不明,疾病表现多样的全身性疾病。脂肪酸(FA)是参与复杂代谢途径的重要生物化合物。它们可能通过参与许多信号通路而导致炎症和内皮功能障碍。FAs水平的增加与各种疾病的风险增加有关。本研究旨在确定FA,BD,和血栓性并发症。从单个中心的风湿病科招募了97名患者作为病例对照研究。参与者分为三组:36例BD伴血栓形成患者(第1组),24例无血栓形成的BD患者(第2组),和37个年龄和性别匹配的对照(第3组)。分析了样品中碳数在(4:0)和(24:1)范围内的37种不同的FA,并进行了组间比较。肉豆蔻酸(MA),二十碳三烯酸甲酯,发现有血栓形成的BD中硬脂酸(STA)水平明显高于无血栓形成的BD,有血栓形成的BD和棕榈酸(PA)水平明显高于健康个体。发现MA是区分血栓性BD的重要标志物。PA和STA是检测血栓性BD的重要标志物。在BD,FA产生的脂毒性,比如PA,STA,还有MA,通过各种机制作为炎症和血栓形成的诱导剂发挥作用。
    Behçet\'s disease (BD) is a systemic disease with unknown etiopathogenesis and varying disease presentations. Fatty acids (FA) are essential biological compounds that are involved in complex metabolic pathways. They may contribute to inflammation and endothelial dysfunction by participating in many signaling pathways. Increased FAs levels are associated with an increased risk for various diseases. This study aimed to determine the relationship between FA, BD, and thrombotic complications. A total of 97 patients were recruited from the rheumatology department of a single center as a case-control study. The participants were divided into three groups: 36 patients with BD with thrombosis (Group 1), 24 patients with BD without thrombosis (Group 2), and 37 age- and sex-matched controls (Group 3). The analysis of 37 different FA with carbon numbers in the range of (4:0) and (24:1) in the samples were analyzed and compared between groups. Myristic acid (MA), methyl eicosatrienoate, and stearic acid (STA) levels were found to be significantly higher in BD with thrombosis than in BD without thrombosis, and palmitic acid (PA) levels were significantly higher in BD with thrombosis than in healthy individuals. MA was found to be a significant marker for differentiating between thrombotic BD. PA and STA are important markers for detecting thrombotic BD. In BD, lipotoxicity created by FA, such as PA, STA, and MA, plays a role as an inducer of inflammation and thrombosis through various mechanisms.
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  • 文章类型: Journal Article
    目的:我们旨在评估临床特征,接受TNF-i治疗的重度/难治性BD患者的病程和预后的相关因素。
    方法:这项回顾性研究是通过回顾土耳其东部凡省三级转诊中心的医疗记录进行的。数据来自2019年6月至2022年6月随访的患者图表。
    结果:我们纳入了469名BD患者(59.3%为男性),其中80名患者(17%)接受了TNF-i治疗。患者的平均值±标准偏差(SD)为36.7±10.1年,中位(IQR)病程为12(12)年。IFX和ADA在67.5%(n=54)和32.5%(n=26)患者中开始,分别。TNF-i的总体和一线保留率IFX分别为84.7%和92.6%,ADA分别为83.3%和80.8%。分别。9例患者因过敏反应和肺结核停用IFX,其中2例患者因过敏反应而停用IFX,3名患者无效,一名心力衰竭患者和一名眼眶带患者。虽然,ADA没有观察到严重的advers事件,5名患者由于无效而改用IFX。总的来说,72名患者(90%)在研究结束时恢复了TNF-i;3名患者(3.8%)因严重ADvers事件而停用TNF-i,5名患者(6.2%)延长了缓解期。
    结论:在我们的研究中,在接受b-DMARD的患者中未观察到死亡病例.大多数患者实现了无发作和无CS的疾病,并保留了b-DMARD治疗。TNF抑制剂对重度/难治性Behçet病患者安全有效。
    OBJECTIVE: We aimed to evaluate the clinical features, disease course, and associated factors for outcome in severe/refractory BD patients receiving TNF-i treatment.
    METHODS: This retrospective study was conducted by reviewing medical records from a tertiary referral center in Van province in Eastern Turkey. Data were obtained from patients\' charts followed up between June 2019 and June 2022.
    RESULTS: We included 469 BD patients (59.3% male) whose 80 patients (17%) received TNF-i treatment in the study. The mean ± standard deviation of the patient age was 36.7 ± 10.1 years and the median (IQR) disease duration was 12 (12) years. IFX and ADAwere initiated in 67.5% (n = 54) and 32.5% (n = 26) patients, respectively. Overall and first-line retention rates of TNF-i were 84.7% and 92.6% for IFX and 83.3% and 80.8% for ADA, respectively. IFX was discontinued in 9 patients which were in 2 patients due to allergic reaction and tuberculosis, 3 patients for inefficacy, one patient for heart failure, and one patient for orbital zona. Although no serious adverse event was observed with ADA, 5 patients switched to IFX due to inefficacy. Overall, 72 patients (90%) resumed TNF-i at the end of the study; TNF-i was discontinued in 3 patients (3.8%) due to severe adverse events and in 5 patients (6.2%) with prolonged remission.
    CONCLUSIONS: In our study, no case of death was observed in TNF-i receiving patients. Most patients achieved attack-free and CS-free disease and retained TNF-i treatment. TNF inhibitors appear to be safe and effective in patients with severe/refractory Behçet\'s disease.
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