polymyalgia rheumatica

风湿性多肌痛
  • 文章类型: Journal Article
    大血管血管炎(LVV)是一组以主动脉及其主要分支炎症为特征的疾病,其中包括巨细胞动脉炎(GCA),风湿性多肌痛(PMR),和大动脉炎(TAK)。这些病症由于其不同的临床表现和潜在的严重并发症而带来重大的诊断和管理挑战。18F-氟代脱氧葡萄糖正电子发射断层扫描计算机断层扫描(18F-FDG-PET-CT)已成为诊断和监测LVV的一种有价值的成像方式,提供对疾病活动的见解,范围,以及对治疗的反应。18F-FDG-PET-CT通过可视化血管受累在LVV的诊断和管理中起着至关重要的作用。评估疾病活动,并指导治疗决策。研究已经证明了18F-FDG-PET-CT在区分LVV亚型中的实用性,评估疾病分布,并检测颅骨GCA或PMR表型患者的颅外受累。此外,18F-FDG-PET-CT在预测临床结果和评估治疗反应方面显示出有希望的效用。基于FDG摄取减少与疾病控制改善之间的相关性。未来的研究应该集中在进一步完善PET-CT技术,探索它们在监测治疗反应中的效用,并研究新的成像方式,如PET-MRI,以提高LVV的诊断准确性。总的来说,18F-FDG-PET-CT是LVV多学科管理的重要工具,促进及时诊断和个性化治疗策略,以改善患者预后。
    Large-vessel vasculitis (LVV) is a group of diseases characterized by inflammation of the aorta and its main branches, which includes giant cell arteritis (GCA), polymyalgia rheumatica (PMR), and Takayasu\'s arteritis (TAK). These conditions pose significant diagnostic and management challenges due to their diverse clinical presentations and potential for serious complications. 18F-fluorodeoxyglucose positron emission tomography-computed tomography (18F-FDG-PET-CT) has emerged as a valuable imaging modality for the diagnosis and monitoring of LVV, offering insights into disease activity, extent, and response to treatment. 18F-FDG-PET-CT plays a crucial role in the diagnosis and management of LVV by allowing to visualize vessel involvement, assess disease activity, and guide treatment decisions. Studies have demonstrated the utility of 18F-FDG-PET-CT in distinguishing between LVV subtypes, evaluating disease distribution, and detecting extracranial involvement in patients with cranial GCA or PMR phenotypes. Additionally, 18F-FDG-PET-CT has shown promising utility in predicting clinical outcomes and assessing treatment response, based on the correlation between reductions in FDG uptake and improved disease control. Future research should focus on further refining PET-CT techniques, exploring their utility in monitoring treatment response, and investigating novel imaging modalities such as PET-MRI for enhanced diagnostic accuracy in LVV. Overall, 18F-FDG-PET-CT represents a valuable tool in the multidisciplinary management of LVV, facilitating timely diagnosis and personalized treatment strategies to improve patient outcomes.
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  • 文章类型: Journal Article
    风湿性多肌痛(PMR)是一种常见于50岁及以上人群的炎症性疾病。这种情况的特征是存在主要涉及肩部和骨盆带的疼痛和僵硬。除了经常与巨细胞动脉炎(GCA)有关,几种情况可能模仿PMR或存在PMR特征。由于诊断基本上是临床的,通常需要对这种情况进行适当的诊断。正电子发射断层扫描/计算机断层扫描(PET-CT)已被证明是诊断PMR的有用工具。18F-FDG-PET成像的使用似乎很有希望,因为它提供了有关炎症活性的详细信息,而传统方法可能不明显。然而,由于PET-CT对于PMR的诊断并不是严格必要的,临床医生应考虑这种成像技术可用于怀疑PMR的患者的几种情况.
    Polymyalgia rheumatica (PMR) is an inflammatory disease common in people aged 50 years and older. This condition is characterized by the presence of pain and stiffness involving mainly the shoulder and pelvic girdle. Besides the frequent association with giant cell arteritis (GCA), several conditions may mimic PMR or present with PMR features. Since the diagnosis is basically clinical, an adequate diagnosis of this condition is usually required. Positron emission tomography/computed tomography (PET-CT) has proved to be a useful tool for the diagnosis of PMR. The use of 18F-FDG-PET imaging appears promising as it provides detailed information on inflammatory activity that may not be evident with traditional methods. However, since PET-CT is not strictly necessary for the diagnosis of PMR, clinicians should consider several situations in which this imaging technique can be used in patients with suspected PMR.
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  • 文章类型: Case Reports
    缓解性血清阴性对称性滑膜炎伴凹陷性水肿是一种罕见的风湿病,以老年男性为主。它的特点是突然出现明显的凹陷性水肿,对称性远端滑膜炎,缺乏类风湿因子和对糖皮质激素的良好反应。RS3PE可能是恶性肿瘤的先兆,因此诊断应迅速评估和排除此类疾病;在这些情况下,对糖皮质激素的反应仅是部分的,治疗瘤形成至关重要。鉴别诊断包括迟发性类风湿关节炎,风湿性多肌痛和焦磷酸钙晶体相关关节炎。我们介绍了一例缓解血清阴性对称性滑膜炎伴与透明细胞肾细胞癌相关的凹陷性水肿的患者。
    Remitting seronegative symmetrical synovitis with pitting oedema is a rare rheumatological condition, predominating in the elderly male. It is characterised by the abrupt onset of marked pitting oedema, symmetrical distal synovitis, absence of rheumatoid factor and an excellent response to glucocorticoids. RS3PE may be the harbinger of a malignancy so the diagnosis should prompt evaluation and exclusion of such condition; in these cases, the response to glucocorticoids is only partial and treating the neoplasia is essential. The differential diagnosis includes late-onset rheumatoid arthritis, polymyalgia rheumatica and calcium pyrophosphate crystal-related arthritis. We present the case of a patient with remitting seronegative symmetrical synovitis with pitting oedema associated with clear cell renal cell carcinoma.
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  • 文章类型: Journal Article
    DANIsh血管炎队列研究,DANIVAS,是一项观察性的国家多中心研究,其总体目标是从丹麦风湿病科诊断和/或随访的风湿性多肌痛(PMR)和巨细胞动脉炎(GCA)患者中前瞻性地收集原型化的临床数据和生物库材料。长期的关键目标是研究使用新的临床实施的诊断成像方式是否有助于GCA-PMR疾病谱中的疾病分层。特别是,我们的目标是评估有和没有大血管受累的GCA患者的治疗要求,有和没有亚临床巨细胞动脉炎的PMR患者的治疗需求,以及影像学对动脉瘤发展的预后作用。因此,在GCA和PMR中,假设影像分层能够指导管理策略。在丹麦建立了风湿病学临床研究的基础设施,丹麦风湿病生物库的基础设施,以及将数据与有效的全国注册管理机构交叉链接的可能性,DANIVAS项目具有收集有关诊断的全面实际数据的特殊可能性,疾病严重程度,疾病持续时间,治疗效果,并发症,和不良事件。在本文中,我们提出了DANIVAS研究的研究方案.临床试验注册:https://clinicaltrials.gov/,标识符NCT05935709。
    The DANIsh VASculitis cohort study, DANIVAS, is an observational national multicenter study with the overall aim to prospectively collect protocolized clinical data and biobank material from patients with polymyalgia rheumatica (PMR) and giant cell arteritis (GCA) diagnosed and/or followed at Danish rheumatology departments. A long-term key objective is to investigate whether the use of new clinically implemented diagnostic imaging modalities facilitates disease stratification in the GCA-PMR disease spectrum. In particular, we aim to evaluate treatment requirements in GCA patients with and without large-vessel involvement, treatment needs in PMR patients with and without subclinical giant cell arteritis, and the prognostic role of imaging with respect to aneurysm development. Hence, in GCA and PMR, imaging stratification is hypothesized to be able to guide management strategies. With an established infrastructure within rheumatology for clinical studies in Denmark, the infrastructure of the Danish Rheumatologic Biobank, and the possibility to cross-link data with valid nationwide registries, the DANIVAS project holds an exceptional possibility to collect comprehensive real-world data on diagnosis, disease severity, disease duration, treatment effect, complications, and adverse events. In this paper, we present the research protocol for the DANIVAS study. Clinical trial registration: https://clinicaltrials.gov/, identifier NCT05935709.
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  • 文章类型: Journal Article
    一名50岁的有局限性皮肤硬皮病病史的患者开始患有多关节痛(左肩,肘部和臀部)无僵硬或相关的炎症综合征。怀疑周围性脊柱关节炎有部分反应,开始口服抗炎药治疗。这随着臀部僵硬和功能受限的出现以及发病后两周炎症综合征的增加而发展。决定进行与风湿性多肌痛兼容的18F-FDG-PET扫描。患者接受口服皮质类固醇治疗,治疗一周后反应良好。
    结论:应考虑风湿性多肌痛,即使是年轻人,临床表现不典型。在考虑诊断风湿病多肌痛之前,应排除感染后和副肿瘤性炎性风湿病。18F-FDG-PET在PMR的阳性诊断和鉴别诊断中起重要作用。
    A 50-year-old patient with a history of limited cutaneous scleroderma began with polyarthralgia (left shoulder, elbows and hips) without stiffness or associated inflammatory syndrome. Treatment with oral anti-inflammatory drugs was started on suspicion of peripheral spondyloarthritis with partial response. This progressed with the appearance of stiffness and functional limitation of the hips as well as an increase in the inflammatory syndrome two weeks after onset. It was decided to perform an 18F-FDG-PET scan compatible with polymyalgia rheumatica. The patient was treated with oral corticosteroids with an excellent response after one week of treatment.
    CONCLUSIONS: Polymyalgia rheumatica should be considered, even in young adults, with atypical clinical presentation.Post-infectious and paraneoplastic inflammatory rheumatism should be ruled out before considering the diagnosis of polymyalgia rheumatica.18F-FDG-PET plays an important role in the positive diagnosis of PMR and in the differential diagnosis.
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  • 文章类型: Journal Article
    风湿性多肌痛(PMR)是一种慢性炎症性疾病,可导致近端关节僵硬和疼痛,包括肩膀,臀部和脖子风湿性多肌痛的确切原因尚未完全了解,但是研究表明,遗传和环境因素都可能导致这种情况。先前的研究已将风湿性多肌痛症状的发作和复发与流感和COVID-19疫苗联系起来。美国食品和药物管理局批准了呼吸道合胞病毒(RSV)疫苗,这是一种重组蛋白疫苗,适用于2023年5月的60岁以上的成年人。以前没有关于风湿性多肌痛发作或复发的报道与RSV疫苗有关。人类蛋白质组与RSVF抗原共享一些肽,提示在疫苗接种制剂中使用该抗原时交叉反应的高风险。
    一名72岁男子在接受Abrysvo®RSV疫苗3天后出现新的双侧肩痛和僵硬。症状持续一个多小时(直到中午),干扰了他的日常生活活动。炎症标志物如C反应蛋白升高。泼尼松治疗后患者的症状和炎症标志物水平显著改善。
    在具有典型PMR症状的患者中,重要的是,临床医生仔细审查免疫史,以排除任何潜在相关的不良反应。
    结论:疫苗可在某些个体中引发自身免疫性疾病。该病例报告表明,呼吸道合胞病毒(RSV)疫苗是风湿性多肌痛的可能诱因之一。
    UNASSIGNED: Polymyalgia rheumatica (PMR) is a chronic inflammatory disorder that causes stiffness and pain in the proximal joints, including the shoulders, hips and neck. The exact cause of polymyalgia rheumatica is yet to be fully understood, but research suggests that both genetic and environmental factors may contribute to it. Studies have previously linked the onset and relapse of polymyalgia rheumatica symptoms to the influenza and COVID-19 vaccines. The Food and Drug Administration approved the respiratory syncytial virus (RSV) vaccine, which is a recombinant protein vaccine for adults over 60, in May 2023. No previous reports of polymyalgia rheumatica onset or relapse have been linked to the RSV vaccine. The human proteome shares some peptides with the RSV F antigen, suggesting a high risk of cross-reactivity when using that antigen in vaccination formulations.
    UNASSIGNED: A 72-year-old man experienced a new onset of bilateral shoulder pain and stiffness three days after receiving the Abrysvo® RSV vaccine. The symptoms lasted more than an hour (up until noon) and interfered with his activities of daily living. Inflammatory markers such as C-reactive protein were elevated. The patient\'s symptoms and inflammatory marker levels significantly improved with prednisone therapy.
    UNASSIGNED: In patients with typical PMR symptoms, it is important for clinicians to carefully review immunisation history to rule out any potentially related adverse effects.
    CONCLUSIONS: Vaccines can trigger autoimmune diseases in some individuals.This case report suggests respiratory syncytial virus (RSV) vaccine is among the possible triggers for polymyalgia rheumatica.
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  • 文章类型: Case Reports
    在这个案例报告中,我们重点介绍了一例罕见的回文风湿病(PR),表现为风湿性多肌痛(PMR)。许多挑战和复杂性与PR的诊断和治疗相关。文献综述仅显示了这种独特表现的少数病例报告。公关有一个独特的表现,经常被忽视,并被医疗专业人员误解。需要更彻底的临床方法来识别和治疗这种情况。我们希望分享这种罕见的病例将有助于医学界更好地了解PR并开发改进的诊断和治疗方案。此病例还表明需要进一步研究以更好地了解这种罕见疾病的发病机理。
    In this case report, we highlight a rare case of palindromic rheumatism (PR) presenting as polymyalgia rheumatica (PMR). Many challenges and complexities are associated with diagnosing and treating PR. Literature reviews showed only a few case reports of this unique presentation. PR has a distinct presentation that often goes unnoticed and is misinterpreted by medical professionals. A more thorough clinical approach is required to identify and treat this condition. We hope sharing such uncommon cases will help the medical community better understand PR and develop improved diagnostic and therapeutic options. This case also demonstrates the need for further research to better understand the pathogenesis of this uncommon condition.
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  • 文章类型: Journal Article
    巨细胞动脉炎(GCA)是一种主要影响老年人的大血管血管炎。然而,由于GCA在东亚的患病率极低,因此有关韩国GCA患者的数据很少.本研究旨在探讨韩国GCA患者的临床特征及其转归。专注于复发。
    回顾性回顾了2007年至2022年在三家三级医院接受治疗的27例GCA患者的病历。
    17名(63.0%)患者为女性,诊断时的中位年龄为75岁.12例(44.4%)患者检测到大血管受累(LVI),14例(51.9%)患者存在风湿性多肌痛(PMR)。12例(44.4%)患者在发病时出现发热。诊断时LVI或并发PMR的存在与C反应蛋白水平(p=0.039)或红细胞沉降率(p=0.034)正常化的时间更长有关。随访期间(中位数33.8个月),4例(14.8%)患者出现复发.Kaplan-Meier分析显示,复发与视力丧失(p=0.008)和无发热(p=0.004)有关。但没有LVI或并发PMR。
    在大约一半的韩国GCA患者中观察到PMR和LVI并发,这些患者的炎症标志物恢复正常所需的时间更长。韩国GCA复发率低于西方国家,和无脑患者或在发病时视力丧失的患者有较高的复发风险,建议医生应仔细监测具有这些特征的患者。
    UNASSIGNED: Giant cell arteritis (GCA) is a large-vessel vasculitis that primarily affects elderly individuals. However, data regarding Korean patients with GCA are scarce owing to its extremely low prevalence in East Asia. This study aimed to investigate the clinical characteristics of Korean patients with GCA and their outcomes, focusing on relapse.
    UNASSIGNED: The medical records of 27 patients with GCA treated at three tertiary hospitals between 2007 and 2022 were retrospectively reviewed.
    UNASSIGNED: Seventeen (63.0%) patients were females, and the median age at diagnosis was 75 years. Large vessel involvement (LVI) was detected in 12 (44.4%) patients, and polymyalgia rheumatica (PMR) was present in 14 (51.9%) patients. Twelve (44.4%) patients had fever at onset. The presence of LVI or concurrent PMR at diagnosis was associated with a longer time to normalization of the C-reactive protein level (p=0.039) or erythrocyte sedimentation rate (p=0.034). During follow-up (median 33.8 months), four (14.8%) patients experienced relapse. Kaplan-Meier analyses showed that relapse was associated with visual loss (p=0.008) and the absence of fever (p=0.004) at onset, but not with LVI or concurrent PMR.
    UNASSIGNED: Concurrent PMR and LVI were observed in approximately half of Korean patients with GCA, and the elapsed time to normalization of inflammatory markers in these patients was longer. The relapse rate in Korean GCA is lower than that in Western countries, and afebrile patients or patients with vision loss at onset have a higher risk of relapse, suggesting that physicians should carefully monitor patients with these characteristics.
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  • 文章类型: Journal Article
    Treatment of giant cell arteritis (GCA) aims initially to prevent acute visual loss, and subsequently to optimise long-term quality of life. Initial prevention of acute visual loss in GCA is well-standardised with high-dose glucocorticoid therapy but in the longer term optimising quality of life requires tailoring of treatment to the individual. The licensing of the IL-6 receptor inhibitor tocilizumab combined with advances in vascular imaging have resulted in many changes to diagnostic and therapeutic practice. Firstly, GCA is a systemic disease that may involve multiple vascular territories and present in diverse ways. Broadening of the \"spectrum\" of what is called GCA has been crystallised in the 2022 GCA classification criteria. Secondly, the vascular inflammation of GCA frequently co-exists with the extracapsular musculoskeletal inflammation of the related disease, polymyalgia rheumatica (PMR). Thirdly, GCA care must often be delivered across multiple specialities and healthcare organisations requiring effective interprofessional communication. Fourthly, both GCA and PMR may follow a chronic or multiphasic disease course; long-term management must be tailored to the individual patient\'s needs. In this article we focus on some areas of current rheumatology practice that ophthalmologists need to be aware of, including comprehensive assessment of extra-ocular symptoms, physical signs and laboratory markers; advanced imaging techniques; and implications for multi-speciality collaboration.
    摘要: 巨细胞动脉炎(GCA)最初治疗的目的是预防急性视力丧失, 随后为改善远期生活质量。大剂量糖皮质激素可较好地标准化GCA患者急性视力丧失的初步预防, 但改善远期生活质量需要制定个体化治疗方案。IL-6受体抑制剂托珠单抗的批准以及活体血管成像的进步, 改变了GCA的诊疗模式。首先, GCA是一种全身性疾病, 累及多个血管区域并且表型多样。在2022年制定的GCA分类标准中, 拓宽了所谓的GCA的“范围”。其次, GCA的血管炎症经常与相关疾病风湿性多肌痛(PMR)的包膜外肌肉骨骼炎症共存。第三, GCA的护理通常须跨多个专科并且医疗机构需要有效的跨专业沟通。第四, GCA和PMR都可能符合慢性或多相病程;长期管理必须根据每个病人的需要制定个体化治疗方案。本文重点介绍了眼科医生需要了解的当前风湿病临床诊疗领域中的一些知识, 包括对眼外症状、体征和实验室标志物的全面评估;先进的影像诊断技术;以及多学科科协作的重要意义。.
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  • 文章类型: Journal Article
    风湿性多肌痛(PMR)是一种病因不明的炎症性疾病,巨细胞关节炎(GCA)和类风湿性关节炎(RA)共同症状。致病性炎症的根源仍然没有很好地理解,并且缺乏广泛的生物标志物研究来解释疾病的首次出现和急性期。本研究旨在深入分析糖皮质激素治疗前后PMR患者的血清蛋白质组和炎性反应。我们纳入了未接受治疗的PMR患者,在治疗前和治疗后3个月收集样本。为了比较,纳入疾病缓解抗风湿药(DMARD)-初治RA患者,并与健康对照(CTL)相匹配.血清蛋白质组使用无标记定量质谱检查,而使用多重炎性细胞因子和无细胞DNA测定评估炎症水平。四组的血清蛋白质组包括急性期反应物,凝血因子,补体蛋白,免疫球蛋白,和载脂蛋白。通过主动PMR治疗,血清淀粉样蛋白A(SAA1)显着降低。由于急性炎症,PMR和RA组的无细胞DNA水平明显高于健康对照组。补体因子在治疗后变化最小。PMR患者的个体血清蛋白质组显示超过100个丰富的可变蛋白质,强调PMR疾病首次亮相的系统性影响和治疗效果。白细胞介素(IL)-6和干扰素-γ(IFN-γ)受到糖皮质激素治疗的显着影响。我们的研究定义了糖皮质激素治疗期间的PMR血清蛋白质组,并强调了SAA1,IL-6和IFN-γ在治疗反应中的作用。PGLYRP2参与急性PMR可能表明对细菌感染的反应,强调其在免疫反应急性期的作用。结果表明,PMR可能是对细菌感染的异常反应,具有加剧的IL-6和急性期炎症反应以及限制炎症的分子尝试。
    Polymyalgia rheumatica (PMR) is an inflammatory disorder of unknown etiology, sharing symptoms with giant cell arthritis (GCA) and rheumatoid arthritis (RA). The pathogenic inflammatory roots are still not well understood, and there is a lack of extensive biomarker studies to explain the disease debut and post-acute phase. This study aimed to deeply analyze the serum proteome and inflammatory response of PMR patients before and after glucocorticoid treatment. We included treatment-naïve PMR patients, collecting samples before and after 3 months of treatment. For comparison, disease-modifying antirheumatic drug (DMARD)-naïve RA patients were included and matched to healthy controls (CTL). The serum proteome was examined using label-free quantitative mass spectrometry, while inflammation levels were assessed using multiplex inflammatory cytokine and cell-free DNA assays. The serum proteomes of the four groups comprised acute phase reactants, coagulation factors, complement proteins, immunoglobulins, and apolipoproteins. Serum amyloid A (SAA1) was significantly reduced by active PMR treatment. Cell-free DNA levels in PMR and RA groups were significantly higher than in healthy controls due to acute inflammation. Complement factors had minimal changes post-treatment. The individual serum proteome in PMR patients showed over 100 abundantly variable proteins, emphasizing the systemic impact of PMR disease debut and the effect of treatment. Interleukin (IL)-6 and interferon-gamma (IFN-γ) were significantly impacted by glucocorticoid treatment. Our study defines the PMR serum proteome during glucocorticoid treatment and highlights the role of SAA1, IL-6, and IFN-γ in treatment responses. An involvement of PGLYRP2 in acute PMR could indicate a response to bacterial infection, highlighting its role in the acute phase of the immune response. The results suggest that PMR may be an aberrant response to a bacterial infection with an exacerbated IL-6 and acute phase inflammatory response and molecular attempts to limit the inflammation.
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