neuroimmunological disorders

神经免疫疾病
  • 文章类型: Journal Article
    目的虽然神经免疫疾病的患者往往需要接受糖皮质激素治疗,并且存在发生糖皮质激素诱导的骨质疏松的风险。目前还没有研究关注糖皮质激素诱导的骨质疏松患者的治疗.方法我们比较了地诺单抗和双膦酸盐在神经免疫疾病中糖皮质激素诱导的骨质疏松症中的疗效。在57例接受皮质类固醇治疗的神经免疫疾病患者中(34例患有视神经脊髓炎谱系疾病,16患有重症肌无力,和7与其他人),我们回顾性研究了通过双能X线吸收法测量的地诺单抗(n=23)和双膦酸盐(n=34)对脊柱和全髋骨矿物质密度(BMD)的长期影响.结果两组患者年龄差异无统计学意义,腰椎BMD,或在基线时替诺塞麦和双膦酸盐组之间的泼尼松龙给药的平均剂量或持续时间。在长达6年的随访期间,denosumab组腰椎和全髋部BMD的增加大于双膦酸盐组(p<0.01)。在denosumab组的23例患者中有2例(9%)和在双膦酸盐组的34例患者中有2例(6%)观察到骨折不足(不明显)。结论Denosumab在增加接受糖皮质激素的神经免疫障碍患者的BMD方面比二膦酸盐更有效。
    Objective Although patients with neuroimmunological disorders often need to be treated with glucocorticoids and are at risk of developing glucocorticoid-induced osteoporosis, no research has focused on the treatment of glucocorticoid-induced osteoporosis in such patients. Methods We compared the efficacy of denosumab and bisphosphonates in glucocorticoid-induced osteoporosis in neuroimmunological diseases. In 57 patients with neuroimmunological disorders treated with corticosteroids (34 with neuromyelitis optica spectrum disorders, 16 with myasthenia gravis, and 7 with others), we retrospectively studied the long-term effects of denosumab (n=23) and bisphosphonates (n=34) on spine and total hip bone mineral density (BMD) measured by dual energy X-ray absorptiometry. Results There were no significant differences in the age, lumbar spine BMD, or mean dose or duration of prednisolone administration at baseline between the denosumab and bisphosphonate groups. During the follow-up period of up to 6 years, the increase in the lumbar spine and total hip BMD was greater in the denosumab group than in the bisphosphonate group (p<0.01). Insufficient bone fractures were observed in 2 (9%) of the 23 patients in the denosumab group and in 2 (6%) of the 34 patients in the bisphosphonate group (not significant). Conclusion Denosumab is more effective than bisphosphonates in increasing the BMD of patients with neuroimmunological disorders receiving glucocorticoids.
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  • 文章类型: Journal Article
    治疗性血浆置换(TPE)是一种血浆置换程序,其儿科神经免疫疾病(PNID)的安全性数据有限。本研究记录了TPE在这些条件下的安全性和可行性数据。
    本研究涉及6组接受TPE的PNID患者:神经脊髓炎视谱障碍(NMOSD),自身免疫性脑炎(AIE),急性播散性脑脊髓炎(ADEM),多发性硬化症(MS),格林-巴利综合征(GBS)和视神经炎(开)。该研究记录了与每个TPE过程相关的并发症。此外,在这些患者中研究了TPE的疗效。
    本研究记录了18名接受121个TPE周期的PNID患者的不良反应:MS中的5个周期(4.13%),AIE亚组中有三人(2.48%),ADEM中的一个(0.82%),和两个(1.65%)在GBS中。所有患者均未出现严重并发症。
    PNID患者耐受治疗性血浆置换,这是一个安全的过程。
    UNASSIGNED: Therapeutic plasma exchange (TPE) is a plasmapheresis procedure whose Safety data for pediatric neuro-immunological disorders (PNID) is confined. The present research documents TPE\'s safety and feasibility data in these conditions.
    UNASSIGNED: The current study involved six distinct groups of patients with PNID undergoing TPE: neuromyelitis optic spectrum disorder (NMOSD), autoimmune encephalitis (AIE), acute disseminated encephalomyelitis (ADEM), multiple sclerosis (MS), Guillain-Barre syndrome (GBS), and optic neuritis (ON). This study documented complications related to each TPE process. In addition, TPE\'s efficacy was studied in these patients.
    UNASSIGNED: The present study recorded adverse effects in 18 patients with PNID that received 121 TPE cycles: five cycles (4.13%) in MS, three (2.48%) in AIE subgroup, one (0.82%) in ADEM, and two (1.65%) in GBS. No severe complications were observed among the patients.
    UNASSIGNED: Patients with PNID tolerated therapeutic plasma exchange, which was a safe process.
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  • 文章类型: Journal Article
    背景:治疗性血浆置换(TPE)在东南亚地区发挥了重要作用。这项研究调查了在该地区进行TPE的挑战。
    方法:2021年1月对来自7个国家的15名东南亚治疗性血浆置换联盟(SEATPEC)成员进行了问卷调查。它包括人口统计,TPE技术,适应症,挑战,定时,结果测量,并在每个当地中心进行实验室测试。
    结果:共纳入来自12个参与中心的15名神经科医师。他们通常进行五次TPE(100.0%),血浆体积为1至1.5(93.3%),和通过中心导管交换(100.0%)。视神经脊髓炎谱系障碍和重症肌无力的急性复发是最常见的适应症。他们使用生理盐水和5%白蛋白(60.0%)的组合作为替代液。大多数(66.7%)使用TPE作为类固醇难治性病例的附加治疗或作为严重发作的一线治疗。他们建议通过下一次发作的间隔时间来评估TPE的疗效,TPE后复发率,和TPE相关并发症。我们地区的主要挑战是费用,报销,和访问TPE。
    结论:尽管各国之间存在差异,所有人在方法上都有相似之处,适应症,定时,障碍,以及TPE对神经免疫疾病的挑战。区域合作对于确定减少未来获得TPE的这些障碍的战略至关重要。
    BACKGROUND: Therapeutic plasma exchange (TPE) for neuroimmunological disorders has played an important role in the Southeast Asian region. This study investigates the challenges of performing TPE within the region.
    METHODS: A questionnaire-based survey was conducted and launched to 15 South East Asian Therapeutic Plasma Exchange Consortium (SEATPEC) members from seven countries in January 2021. It included demographics, TPE techniques, indications, challenges, timing, outcome measurement, and access to laboratory testing in each local center.
    RESULTS: A total of 15 neurologists from 12 participating centers were included. They usually perform five sessions of TPE (100.0%), with 1 to 1.5 plasma volume (93.3%), and exchanges via the central catheter (100.0%). Acute relapses of neuromyelitis optica spectrum disorder and myasthenia gravis are the most common indications. They used a combination of normal saline and 5% albumin (60.0%) as replacement fluid. Most (66.7%) used TPE as an add-on treatment in steroid-refractory cases or as first-line treatment for severe attacks. They suggested assessing the TPE efficacy of TPE by the interval to the next attack, post-TPE relapse rates, and TPE-related complications. The major challenges within our region are expense, reimbursibility, and access to TPE.
    CONCLUSIONS: Although countrywise differences exist, all share similarities regarding methods, indications, timing, obstacles, and challenges of TPE for neuroimmunological conditions. Regional collaboration will be essential to identify strategies to reduce these barriers to access to TPE in the future.
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  • 文章类型: Journal Article
    神经免疫疾病是神经系统的疾病,其中免疫系统有助于组织损伤和修复。自身抗体是诊断神经免疫疾病和评估疾病活动的有用生物标志物。新出现的证据表明几种自身抗体与神经免疫疾病相关。虽然已经建立了基于自身抗体阳性的鉴别诊断过程,这些自身抗体产生的潜在机制仍需研究.自身抗体不一定是致病的,和一些参与免疫调节。产生自身抗体的成浆细胞参与疾病的致病性和免疫调节。因此,这些致病性和调节性浆细胞之间的比较可能为我们提供线索,了解自身抗体相关的神经免疫疾病的机制。此外,阐明这些机制可能有助于开发新的免疫调节疗法,以促进神经免疫疾病中的调节性B细胞功能。为此,本文讨论了成浆细胞在神经免疫疾病中的作用。
    Neuroimmunological disorders are diseases of the nervous system, wherein the immune system contributes to tissue injury and repair. Autoantibodies are useful biomarkers for the diagnosis of neuroimmunological disorders and evaluating disease activity. Emerging evidence indicates that several autoantibodies are associated with neuroimmunological diseases. While the differential diagnostic process based on the positivity of autoantibodies has been established, the mechanisms underlying the production of these autoantibodies still need to be investigated. Autoantibodies are not necessarily pathogenic, and some are involved in immune regulation. Autoantibody-producing plasmablasts are involved in both pathogenicity and immune regulation of diseases. Thus, comparisons between these pathogenic and regulatory plasmablasts may give us clues understanding the machinery of autoantibody-related neuroimmunological diseases. Moreover, elucidating these mechanisms may allow the development of new immune-modulatory therapies to facilitate regulatory B cell function in neuroimmunological diseases. To this end, herein the roles of plasmablasts in neuroimmunological disorders are discussed.
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