HLA B27

HLA B27
  • 文章类型: Journal Article
    JIA是一种具有不同免疫学特征和复杂遗传基础的疾病。HLAB27是JIA发展的危险因素,其对该疾病的免疫发病机制的影响也是一个感兴趣的领域。确定JIA患者和健康人群之间的HLAB27和免疫标志物是否不同。
    这项比较横断面研究是在健康科学大学(UHS)免疫学系进行的,拉合尔从2018年2月到2021年8月。共纳入(71)JIA患者和(34)健康对照。通过流式细胞术计数B细胞,ELISA法检测血清细胞因子水平,SPSS聚合酶链反应法检测HLA-B27等位基因。
    对照组的HLAB27等位基因明显高于患者组,表明它是一个保护等位基因来阻止JIA.HLAB27阳性组的外周血B细胞计数和百分比显着低于对照组的HLAB27阴性组。两个研究人群的HLAB27阳性和HLAB27阴性等位基因之间的血清细胞因子水平没有显着差异。
    在本研究中,B细胞在两组对照群体之间是不同的;血清细胞因子在研究组之间是相当的。不过,提示HLAB27可能是JIA发病的预防性等位基因.
    UNASSIGNED: JIA is a disease with different immunological characteristics and a complicated genetic foundation. HLA B27 is a risk factor for the development of JIA, and its impact on immunopathogenesis of the disease is also an area of interest. To determine whether HLA B27 and immune markers varied between JIA patients and healthy population.
    UNASSIGNED: This comparative cross-sectional study was conducted at Immunology Department of University of Health sciences (UHS), Lahore from February 2018 till August 2021. A total of (71) JIA patients and (34) healthy controls were enrolled. B cells were enumerated by flowcytometry, ELISA was used for serum cytokines estimation and HLA B27 allele was detected by SPSS polymerase chain reaction.
    UNASSIGNED: The HLA B27 allele was significantly more in the control group than in the patient group, suggesting it is a protective allele to prevent JIA. Peripheral blood B cell counts and percentages were significantly lower in the HLA B27 positive group than in the HLA B27 negative group of control population. Serum cytokine levels were not significantly different between the HLA B27 positive and HLA B27 negative allele of the two study populations.
    UNASSIGNED: In this study B cells are different between the two groups of control population however; serum cytokines are comparable between the study groups. Though, it was indicated that HLA B27 may be a preventive allele in the onset of JIA.
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  • 文章类型: Journal Article
    背景:为了评估幼年特发性关节炎(JIA)患者的长期结局和预后因素,表现为少关节炎,他们接受IAC作为他们疾病的第一种治疗方法。
    方法:我们在卢布尔雅那大学儿童医院进行了回顾性研究,斯洛文尼亚,从2015年1月到2023年5月,JIA儿童,临床上表现为少关节炎,接受关节内皮质类固醇注射(IAC)作为初始治疗。收集患者和治疗数据,根据以后的治疗需要,将结果分为三组:不需要治疗,只需要额外的IAC和全身治疗.最后一组根据bDMARD的要求进一步划分。Log-rank(Mantel-Cox)生存分析比较了不同结果组。
    结果:我们纳入了109例JIA患者,表现为少关节炎(63%为女性),首先接受IAC治疗的人。IAC的平均年龄是8.0岁,4.3年随访。值得注意的是,38.5%的患者在IAC后不需要额外的治疗,而15.5%只需要额外的IAC。全身治疗,主要是甲氨蝶呤(MTX),对45.9%的患者来说是必要的,平均在IAC后7.8个月内开始。在IAC后的平均2.2年内,22%的人开始了生物治疗。注射关节的数量与生物制剂的需求相关。在最后一次随访中,88.9%患有非活动性疾病。ANA阳性(P=0.049,卡方3.89)和HLAB27抗原存在(P=0.050,卡方3.85)与全身治疗的需要相关。一个8岁以上儿童的亚组,与其他患者相比,ANA和HLAB27阴性患者所需的全身治疗(25.8%)和生物治疗(9.6%)明显较少(p=0.050,卡方3.77)。
    结论:需要IAC的少关节JIA患儿中几乎40%没有进展为慢性疾病。年龄更小,ANA阳性,HLAB27的存在是全身治疗的预测因素,而注射关节的数量预测了未来对生物治疗的需求。
    BACKGROUND: To evaluate long-term outcomes and prognostic factors in patients with juvenile idiopathic arthritis (JIA), presenting as oligoarthritis, who received IAC as the first treatment for their disease.
    METHODS: We conducted retrospective study at the University Children\'s Hospital Ljubljana, Slovenia, from January 2015 to May 2023 in children with JIA, clinically presenting as oligoarthritis receiving intra-articular corticosteroid injection (IAC) as the initial treatment. Patient and treatment data were collected, and the outcomes were categorized into three groups based on the later need for therapy: no therapy needed, only additional IAC needed and systemic therapy needed. The last group was further divided based on the requirement of bDMARD. Log-rank (Mantel-Cox) survival analyses compared different outcome groups.
    RESULTS: We included 109 patients with JIA, presenting as oligoarthritis (63% female), who were first treated with IAC. The mean age at IAC was 8.0 years, with a 4.3-year follow-up. Notably, 38.5% of patients did not require additional therapy post-IAC, whereas 15.5% required only additional IAC. Systemic therapy, mainly methotrexate (MTX), was necessary for 45.9% of patients, initiated in average 7.8 months post-IAC. Biologic therapy was initiated in 22% in average 2.2 years post-IAC. Number of injected joints correlated with the need for biologics. At the last follow-up, 88.9% had inactive disease. ANA positivity (P = 0.049, chi square 3.89) and HLA B27 antigen presence (P = 0.050, chi square 3.85) were associated with the need for systemic therapy. A subgroup of children older than 8 years, ANA and HLA B27 negative required significantly less systemic (25.8%) and biologic therapy (9.6%) compared to other patients (p = 0.050, chi square 3.77).
    CONCLUSIONS: Almost 40% of children with oligoarticular JIA requiring IAC did not progress to chronic disease. Younger age, ANA positivity, and HLA B27 presence were predictive factors for systemic therapy, while the number of injected joints predicted the future need for biologic therapy.
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  • 文章类型: Case Reports
    强直性脊柱炎可表现为各种关节外表现。已记录了由于主动脉瘤或主动脉炎引起的血管并发症。然而,与颅内血管动脉瘤相关的报道很少.我们报告了一例HLAB27强直性脊柱炎阳性的年轻男性,关节外牵涉,表现为复发性单侧头痛。他被发现患有未破裂的前交通动脉瘤。脑血管造影证实了这一点,他受到了保守的对待。
    Ankylosing spondylitis can present with various extra-articular manifestations. Vascular complications due to aortic aneurysm or aortitis have been documented. However, an association with intracranial vascular aneurysm is rarely reported. We report a case of a young male with positive HLA B27 ankylosing spondylitis, with extra-articular involvements, presented with recurrent unilateral headache. He was found to have an unruptured anterior communicating artery aneurysm. It was confirmed by a cerebral angiogram, and he was treated conservatively.
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  • 文章类型: Journal Article
    反应性关节炎是一种急性炎症性无菌性关节炎,在遗传易感个体中发生感染过程。它与胃肠道或泌尿生殖道感染有关。反应性关节炎在儿童中很少见。在这次审查中,我们介绍了两个需要生物治疗的指标病例,然后对儿童和青少年的反应性关节炎进行了全面审查,并提出了治疗方法.
    Reactive arthritis is an acute inflammatory aseptic arthritis that is preceded by an infectious process in genetically predisposed individuals. It has been associated with gastrointestinal or genitourinary infection. Reactive arthritis is rare in children. In this review, we present two index cases that need biologic treatment followed by a thorough review of reactive arthritis in children and adolescents with proposed treatment algorithm.
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
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  • 文章类型: Journal Article
    目的:在发展中国家,附着点炎相关关节炎(ERA)的长期功能结局有限。我们评估了预测ERA长期功能结果的临床和遗传因素。
    方法:本横断面研究包括疾病≥5年且≥16岁的ERA患者。从医院记录中收集疾病发作6个月内的临床特征数据。浴指数,HAQ-DI,和WHO-QOL在最后一次访问中进行了评估。不良功能结局(PFO)定义为BASFI>1.5或HAQ-DI>1。持续性疾病活动性(PDA)定义为BASDAI≥4。ERAP-1和IL23RSNP基因分型通过TaqMan方法进行,HLA-B27通过PCR进行。
    结果:181名患者[170名男性,发病年龄12.5(10-15)岁,疾病持续时间7(5-11)年]被招募。诊断延迟3(1-5)年。ASDAS-ESR中位数,BASDAI,HAQ-DI,纳入时的BASFI为2.6(1.8-3.6),2.6(1-5.2),0.5(0-0.5)和1.6(0.3-3.2)。BASFI和HAQ-DI与ASDAS-ESR相关,ASDAS-CRP和WHO-QOL-BREF。PFO患者(n=98)的诊断延迟更长(4年比2年,p<0.001),发病时关节炎的患病率较低(OR=0.3,95CI:0.1-0.8),ERAP1(rs27044)等位基因C的患病率较高(OR=7.2,95CI:1.5-33.7),和目前较高的疾病活动。在多变量分析中,诊断延迟(OR=1.2;95CI:1.08-1.4)是PFO的唯一预测因素。三分之一的患者患有PDA。发病时的关节炎是PDA的唯一预测因子(OR=2.3;95CI:1.009-5.4)。
    结论:长期在一半的JIA-ERA中观察到不良的功能结局,并且与活动性疾病相关,诊断延迟是其唯一的预测指标。
    Long-term functional outcomes in enthesitis-related arthritis (ERA) is limited from developing countries. We assessed the clinical and genetic factors that predicted the long-term functional outcome in ERA.
    Patients with ERA having ≥5 years of disease and >16 years of age were included in this cross-sectional study. Data on clinical features within 6 months of disease onset was collected from hospital records. Bath indices, HAQ Disability Index (HAQ-DI) and World Health Organization\'s Quality of Life (WHO-QOL) were assessed at last visit. Poor functional outcome (PFO) was defined as BASFI > 1.5 or HAQ-DI > 1. Persistent disease activity (PDA) was defined as BASDAI ≥ 4. Endoplasmic reticulum aminopeptidase 1 (ERAP1) and IL-23 receptor single nucleotide polymorphism genotyping was performed with the TaqMan method and HLA-B27 by PCR.
    One hundred and eighty-one patients [170 male, median (interquartile range) age of disease onset 12.5 (10-15) years, disease duration 7 (5-11) years] were recruited. There was a delay in diagnosis of 3 (1-5) years. The median Ankylosing Spondylitis Disease Activity Score (ASDAS)-ESR, BASDAI, HAQ-DI and BASFI at inclusion were 2.6 (1.8-3.6), 2.6 (1-5.2), 0.5 (0-0.5) and 1.6 (0.3-3.2), respectively. BASFI and HAQ-DI correlated with ASDAS-ESR, ASDAS-CRP and WHO-QOL-BREF. Those with PFO (n = 98) had a longer delay in diagnosis (4 vs 2 years, P < 0.001), lower prevalence of arthritis at onset [odds ratio (OR) = 0.3; 95% CI: 0.1, 0.8], higher prevalence of ERAP1 (rs27044) allele C (OR = 7.2; 95% CI: 1.5, 33.7) and higher disease activity currently. Delay in diagnosis (OR = 1.2; 95% CI: 1.08, 1.4) was the sole predictor of PFO in multivariate analysis. One-third of patients had PDA. Tarsitis at disease onset was the sole predictor of PDA (OR = 2.3; 95% CI: 1.009, 5.4).
    PFO was seen in one-half of JIA-ERA in the long-term and was associated with active disease with delay in diagnosis as its sole predictor.
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  • 文章类型: Journal Article
    结膜炎相关关节炎(ERA)是青少年特发性关节炎的一类,属于青少年脊柱关节病中包括的疾病谱。近年来,在理解发病机制方面取得了重大进展,评估疾病活动的工具,轴向疾病的早期识别,以及使用IL-17抑制剂和小分子抑制剂的靶向治疗。当前的叙述审查强调了这些新进展。在将HLAB27与ERA联系起来的许多假设中,其中之一是HLAB27对肠道菌群失调的影响。然而,最近的数据表明,肠道菌群失调可能不是由HLAB27决定的。尽管儿童患有关节炎和附着点炎,MRI显示轴性疾病占50-60%。使用数据驱动的方法,已经为儿童定义了活动性和慢性疾病的鉴别MRI发现。这将有助于疾病的早期识别。不需要急性期反应物和Schober测试以及原始评分的青少年脊柱关节病活动性(JSpADA)评分的删节版可能会增加其在常规实践中的接受度。Secukinumab(抗IL-17抗体)在ERA儿童中显示出超过75%的应答率,可能是抗TNF治疗的良好替代品。托法替尼的初始数据看起来也很有希望。所有这些都将为ERA儿童带来更好的结果。
    Enthesitis-related arthritis (ERA) is a category of juvenile idiopathic arthritis which belongs to the spectrum of diseases that are included in juvenile spondyloarthropathy. In recent years, there have been significant advances in understanding pathogenesis, tools to assess disease activity, early recognition of the axial disease, and targeted therapy using IL-17 inhibitors and small molecule inhibitors. The current narrative review highlights these new advances. Among many hypotheses linking HLA B27 to ERA, one of them is the effect of HLA B27 on gut dysbiosis. However, recent data suggest that gut dysbiosis is probably not determined by HLA B27. Though children present with arthritis and enthesitis, axial disease is present in 50-60% on MRI. Using data-driven approach, discriminative MRI finding for active and chronic diseases has been defined for children. This will help in the early recognition of disease. An abridged version of juvenile spondyloarthropathy disease activity (JSpADA) score without the need for acute phase reactants and Schober test performed as well as the original score may increase its acceptance in routine practice. Secukinumab (anti-IL-17 antibody) has shown a more than 75% response rate in children with ERA and may be a good alternative to anti-TNF therapy. Initial data with tofacitinib also look promising. All these will translate into better outcomes for children with ERA.
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  • 文章类型: Case Reports
    颈动脉夹层是年轻患者中风的重要原因。这里,我们报告了一名33岁的男性,他表现为右侧身体的同义偏盲和感觉异常。大脑的磁共振成像(MRI)显示左侧顶枕区有急性梗塞。颈部血管的磁共振血管造影(MRA)和双工超声检查(USG)提示左颈内动脉夹层是根本原因。该患者是已知的人类白细胞抗原(HLA)B27,并且有葡萄膜炎发作史。此病例报告将提高人们对HLAB27与颈部血管解剖的可能关联的认识。
    Carotid artery dissection is a significant cause of stroke in young patients. Here, we report a 33-year-old male who presented with right homonymous hemianopia and paresthesia of the right side of the body. Magnetic resonance imaging (MRI) of the brain revealed an acute infarct in the left parieto-occipital region. Magnetic resonance angiography (MRA) and duplex ultrasonography (USG) of the neck vessels suggested the left internal carotid artery dissection as the underlying cause. The patient was a known human leukocyte antigen (HLA) B27 and had a history of a previous attack of uveitis. This case report will raise awareness regarding the possible association of HLA B27 with the dissection of neck vessels.
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  • 文章类型: Journal Article
    目的:银屑病,这是一种慢性的,病因不明的免疫介导性皮肤病,不仅影响皮肤,但也与许多全身性疾病有关,如关节炎,心血管疾病,抑郁症,和恶性肿瘤。尽管牛皮癣患者会遇到许多类型的眼睛受累,干眼症是其中的第一位。葡萄膜炎是一种可能与牛皮癣有关的实体,由于晚期诊断可导致严重的视力丧失,不充分和不适当的治疗。在这次审查中,我们的目的是阐明诊断结果,type,通过汇编从已发表的研究中获得的当前数据,对银屑病患者葡萄膜炎的预后和治疗进行分析,并指导这些患者的随访和治疗。
    方法:在PubMed上进行了系统的文献检索,使用关键词包括“牛皮癣”,\"银屑病关节炎\",“葡萄膜炎”,“TNF-抑制剂”,“HLAB27”。
    结果:在文献中,频率,明确定义了银屑病关节炎过程中葡萄膜炎的类型和治疗方法。然而,牛皮癣和葡萄膜炎的共存尚未得到澄清,因为很少有发表的研究和这些研究的设计。由于我们检查了现有的研究,我们确定牛皮癣和葡萄膜炎的共存可能是急性或阴险的,并且葡萄膜炎的概率和严重程度随着皮肤和关节受累的严重程度增加而增加。此外,我们发现牛皮癣相关的葡萄膜炎可以是双侧的,慢性,严重进展和高复发率。
    结论:非关节炎性银屑病与葡萄膜炎的关系尚未完全阐明。治疗这些疾病的医生必须谨慎,并将患有牛皮癣的患者转诊给眼科医生进行定期检查,即使他们没有眼睛症状。另一方面,眼科医生必须小心葡萄膜炎患者的皮肤和关节受累,不能忽视潜在的疾病。
    OBJECTIVE: Psoriasis, which is a chronic, immune-mediated skin disease of unknown etiology, not only affects the skin, but also is linked to many systemic conditions such as arthritis, cardiovascular disease, depression, and malignancy. Although many types of eye involvement are encountered in psoriasis patients, dry eye is the first among them. Uveitis is an entity that can be associated with psoriasis and can cause severe vision loss as a result of late diagnosis, inadequate and inappropriate treatment. In this review, we aimed to shed light on the diagnosis, type, prognosis and treatment of uveitis in psoriasis patients by compiling current datas obtained from published studies and to guide the follow-up and treatment of these patients.
    METHODS: A systematic literature search was done on PubMed using key words including \"psoriasis\", \"psoriatic arthritis\", \"uveitis\", \"TNF- inhibitors\", \"HLA B27\".
    RESULTS: In the literature, the frequency, type and treatment of uveitis developing in the course of psoriatic arthritis are clearly defined. However, the coexistence of psoriasis and uveitis has not yet been clarified due to few numbers published studies and designs of these studies. Since we examined the existing studies, we determined that the coexistence of psoriasis and uveitis could be acute or insidious, and the probability and severity of uveitis increased as the severity of skin and joint involvement increased. In addition, we found that psoriasis-associated uveitis can be bilateral, chronic, severe progression and with a high recurrence rate.
    CONCLUSIONS: The relations between non-arthritic psoriasis and uveitis have not yet been fully elucidated. Physicians who treat these diseases must be cautious, and refer their patients who have psoriasis to an ophthalmologist for periodic examination, even if they do not have eye symptoms. On the other hand, ophthalmologists must be careful in uveitis patients in terms of skin and joint involvement, and must not overlook the underlying disease.
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