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[Vasculitic involvement of the skeletal muscle and the peripheral nervous system: clinical and neuropathologic perspective]. [骨骼肌和周围神经系统血管炎受累:临床和神经病理学视角]。
IF 0.9 4区 医学 Q4 RHEUMATOLOGY Pub Date : 2024-09-24 DOI: 10.1007/s00393-024-01567-y
Nikolas Ruffer, Felix Kleefeld, Marie-Therese Holzer, Martin Krusche, Ina Kötter, Udo Schneider, Werner Stenzel

The peripheral nervous system is a classic target organ in systemic vasculitis. In addition, the skeletal muscle can also be affected. Myalgia, muscle weakness and sensory deficits are typical signs, which can lead to severe functional limitations and impaired of quality of life. Vasculitic involvement of the skeletal muscle (vasculitic myopathy [VM]) and peripheral nerves (vasculitic neuropathy [VN]) occurs predominantly in polyarteritis nodosa and small-vessel vasculitis. VM presents with elevated markers of inflammation and is typically characterized by immobilizing myalgia with normal creatine kinase activity and diffuse or patchy areas of hyperintensity on T2-weighted MRI ("MRI myositis without myositis"). In VN, sensor motor deficits predominantly affect the lower extremity in the area supplied by several peripheral nerves (e.g., mononeuritis multiplex) with acute to subacute history. The histopathological examination of nerve and muscle biopsies is the gold standard for the diagnosis of vasculitic manifestations and has a significant impact on the therapeutic approach.

周围神经系统是全身性血管炎的典型靶器官。此外,骨骼肌也会受到影响。肌痛、肌无力和感觉障碍是典型的症状,可导致严重的功能限制和生活质量下降。骨骼肌(血管性肌病 [VM])和周围神经(血管性神经病 [VN])的血管性受累主要发生在结节性多动脉炎和小血管炎中。结节性多动脉炎表现为炎症指标升高,典型特征是固定性肌痛,肌酸激酶活性正常,T2 加权磁共振成像显示弥漫性或斑片状高密度区("无肌炎的磁共振成像肌炎")。在 VN 中,传感器运动障碍主要影响下肢由多条周围神经供应的区域(如多发性单神经炎),病史为急性至亚急性。神经和肌肉活检组织病理学检查是诊断血管炎表现的金标准,对治疗方法有重要影响。
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引用次数: 0
[News on the treatment of large vessel vasculitis]. [大血管炎治疗新闻]。
IF 0.9 4区 医学 Q4 RHEUMATOLOGY Pub Date : 2024-09-20 DOI: 10.1007/s00393-024-01563-2
Nils Venhoff, Markus Zeisbrich

Large vessel vasculitis, such as giant cell arteritis (GCA) and Takayasu arteritis (TAK) are primarily manifested on large and medium-sized arteries. While GCA mainly affects older people after the 6th decade of life onwards, TAK mainly affects young women under the age of 40 years. Glucocorticoids (GC) are still the standard treatment for both diseases. Refractory courses and relapses in particular often lead to long-term treatment with high cumulative doses of GC, which can lead to increased morbidity and mortality. To date, only the interleukin 6 (IL-6) receptor blocker tocilizumab has been approved for the treatment of GCA. The data on methotrexate and other conventional immunosuppressants are incomplete and in some cases contradictory. The early use of steroid-sparing immunosuppressants is recommended for TAK, although the number of randomized placebo-controlled trials is limited and no steroid-sparing treatment has yet been approved for TAK. For both diseases there is still a great need for modern and safe steroid-sparing treatment that effectively treats vasculitis, prevents damage and enables adequate disease monitoring. This article provides an overview of the current study situation and possible future treatment options for GCA and TAK.

大血管脉管炎,如巨细胞动脉炎(GCA)和高安动脉炎(TAK),主要表现在大中型动脉上。巨细胞动脉炎主要影响 60 岁以后的老年人,而高安动脉炎主要影响 40 岁以下的年轻女性。糖皮质激素(GC)仍然是治疗这两种疾病的标准药物。特别是难治性病程和复发往往需要长期使用大剂量的糖皮质激素,这会导致发病率和死亡率上升。迄今为止,只有白细胞介素 6(IL-6)受体阻断剂托西珠单抗被批准用于治疗 GCA。有关甲氨蝶呤和其他常规免疫抑制剂的数据并不完整,有时甚至相互矛盾。尽管随机安慰剂对照试验的数量有限,而且目前还没有一种节省类固醇的治疗方法被批准用于治疗TAK,但还是建议TAK患者尽早使用节省类固醇的免疫抑制剂。对于这两种疾病,我们仍然亟需能够有效治疗脉管炎、预防损害并进行充分疾病监测的现代、安全的类固醇保留治疗。本文概述了 GCA 和 TAK 的研究现状和未来可能的治疗方案。
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引用次数: 0
[Effects of a multimodal inpatient treatment for patients with fibromyalgia syndrome at the Rhineland-Palatinate Acute Rheumatology Center]. [莱茵兰-法尔茨急性风湿病中心对纤维肌痛综合征患者进行多模式住院治疗的效果]。
IF 0.9 4区 医学 Q4 RHEUMATOLOGY Pub Date : 2024-09-18 DOI: 10.1007/s00393-024-01568-x
Konstantinos Triantafyllias, Veronika Balaklytska, Charlotte Sauer, Matthias Dreher, Andreas Schwarting

Introduction: Fibromyalgia syndrome (FMS) is a complex condition that is often refractory to therapy and is associated with impaired quality of life. In some studies, multimodal rheumatological treatment has been shown to be an effective therapy option for patients with systemic-inflammatory and degenerative rheumatic diseases. However, the effects of this therapeutic approach have not been sufficiently investigated in patients with FMS. Therefore, the aim of this study was to examine the effect of a concise 9‑ to 10-day inpatient multimodal fibromyalgia treatment (MFT) using patient-reported outcomes in a German cohort.

Methods: The effects of MFT were assessed using visual analog scales (VAS) for pain (P) and subjective disease activity (DA), questionnaires measuring everyday functional capacity (Health Assessment Questionnaire [HAQ], Funktions-Fragebogen-Hannover [FFbH, Hannover Functional Ability Questionnaire]), and pharmacotherapy at three time points (Visit 1: beginning of multimodal therapy, Visit 2: end of MFT, and Visit 3: 3 months after Visit 2).

Results: Sixty-one patients were enrolled in the study at the Rhineland-Palatinate Acute Rheumatology Center. Under MFT, a significant improvement in VAS (P) and VAS (DA) was observed between the start and end of treatment (Visit 2 versus Visit 1: median decrease from 7 to 5, p < 0.001, for both VAS [P] and VAS [DA]). Additionally, comparison of the other two assessment points showed a change in VAS (P) (Visit 3 versus Visit 1: median decrease from 7 to 6, p = 0.041, and Visit 3 versus Visit 2: median increase from 5 to 6, p = 0.004). However, there were no significant differences in FFbH and HAQ parameters among the three visits. Examination of the subgroup of patients whose medication therapy was not intensified during hospitalization also showed significant improvements in VAS (P) and VAS (DA) between the start and end of MFB (Visit 2 versus Visit 1: median decrease from 7 to 4, p < 0.001, for VAS [P] and median decrease from 6.25 to 4, p = 0.002, for VAS [DA]).

Conclusion: These findings indicate a demonstrable benefit to patients of MFT regarding both pain and subjective disease activity. Furthermore, pain relief was even observed 3 months after the end of therapy. This shows the high value of this therapeutic approach to treating patients with FMS.

导言:纤维肌痛综合征(FMS)是一种复杂的病症,通常难治,且与生活质量受损有关。在一些研究中,多模式风湿治疗已被证明是全身炎症性和退行性风湿病患者的有效治疗方案。然而,这种治疗方法对 FMS 患者的影响尚未得到充分研究。因此,本研究的目的是在德国队列中使用患者报告的结果来检验为期 9-10 天的简易纤维肌痛住院多模式治疗(MFT)的效果:方法:采用疼痛(P)和主观疾病活动度(DA)视觉模拟量表(VAS)、日常功能能力问卷(健康评估问卷[HAQ]、汉诺威功能能力问卷[FFbH])和药物疗法,在三个时间点(第1次就诊:多模式疗法开始;第2次就诊:多模式疗法结束;第3次就诊:第2次就诊后3个月)评估多模式疗法的效果:莱茵兰-法尔茨急性风湿病中心的 61 名患者参加了这项研究。在 MFT 治疗中,观察到 VAS(P)和 VAS(DA)在治疗开始和结束时均有明显改善(第 2 次就诊与第 1 次就诊相比:中位数从 7 降至 5,P 结论:这些结果表明,MFT 治疗对患者有明显的益处:这些研究结果表明,MFT 可使患者在疼痛和主观疾病活动方面明显受益。此外,在治疗结束 3 个月后,患者的疼痛症状也得到了缓解。这表明这种治疗方法对治疗 FMS 患者具有很高的价值。
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引用次数: 0
[News on the imaging of large vessel vasculitis]. [关于大血管炎成像的新闻]。
IF 0.9 4区 医学 Q4 RHEUMATOLOGY Pub Date : 2024-09-13 DOI: 10.1007/s00393-024-01565-0
Valentin S Schäfer, Simon M Petzinna, Wolfgang A Schmidt

Large vessel vasculitis, including giant cell arteritis (GCA) and Takayasu arteritis (TAK), are autoimmune diseases primarily affecting the aorta and its branches. GCA is the most common primary vasculitis. Inflammatory changes in the vessel walls can cause serious complications such as amaurosis, stroke, and aortic dissection and rupture. Imaging techniques have become an integral part for the diagnosis and monitoring of large vessel vasculitis, allowing for effective disease monitoring. GCA and TAK exhibit similar patterns of vascular distribution. However, the temporal arteries are never involved in TAK, and axillary arteritis occurs more frequently in GCA. In most centers, ultrasound of the temporal and axillary arteries has replaced temporal artery biopsy as the primary diagnostic tool for GCA. In addition to ultrasound, magnetic resonance imaging (MRI), computed tomography (CT), and [18F]-FDG (fluorodeoxyglucose) positron emission tomography-computed tomography (PET) are important, particularly for visualizing the aorta. Moreover, PET-CT is now also capable of assessing the temporal arteries, although it is not yet widely available. In polymyalgia rheumatica (PMR), ultrasound of the shoulder and hip regions is part of the ACR/EULAR classification criteria. MRI allows detailed visualization of additional inflammatory extraarticular manifestations, showing characteristic inflammatory lesions in entheses, tendons, and ligaments. [18F]-FDG-PET-CT also enables the visualization of musculoskeletal inflammation, especially in the shoulder and hip regions, as well as paravertebral areas. Ultrasound can detect subclinical GCA in up to 23% of patients with PMR, which should be treated like GCA. Technological innovations such as new radiotracers and improved MRI imaging could further enhance the diagnosis and monitoring of large vessel vasculitis and PMR, thus playing a crucial role in improving the prognosis through faster initiation of therapy.

大血管脉管炎,包括巨细胞动脉炎(GCA)和高安动脉炎(TAK),是主要影响主动脉及其分支的自身免疫性疾病。GCA 是最常见的原发性血管炎。血管壁的炎症变化可导致严重的并发症,如无脑、中风、主动脉夹层和破裂。成像技术已成为诊断和监测大血管炎不可或缺的一部分,可对疾病进行有效监测。GCA 和 TAK 表现出相似的血管分布模式。但是,TAK从未累及颞动脉,而腋动脉炎在GCA中更常发生。在大多数中心,颞动脉和腋动脉的超声检查已取代颞动脉活检,成为 GCA 的主要诊断工具。除超声波外,磁共振成像(MRI)、计算机断层扫描(CT)和[18F]-FDG(氟脱氧葡萄糖)正电子发射断层扫描-计算机断层扫描(PET)也很重要,尤其是在观察主动脉方面。此外,PET-CT 现在也能对颞动脉进行评估,但尚未广泛应用。在多发性风湿痛(PMR)中,肩部和髋部的超声波检查是 ACR/EULAR 分类标准的一部分。核磁共振成像可详细观察到关节外的其他炎症表现,显示粘膜、肌腱和韧带的特征性炎性病变。[18F]-FDG-PET-CT也能显示肌肉骨骼炎症,尤其是肩部和髋部以及椎旁区域。超声波可在多达23%的PMR患者中检测出亚临床GCA,这种情况应像GCA一样进行治疗。新的放射性钙离子和改进的核磁共振成像等技术创新可进一步加强对大血管炎和 PMR 的诊断和监测,从而通过更快地开始治疗在改善预后方面发挥重要作用。
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引用次数: 0
[Digital health applications-What we should know as rheumatologists]. [数字医疗应用--作为风湿病学家我们应该知道什么]。
IF 0.9 4区 医学 Q4 RHEUMATOLOGY Pub Date : 2024-09-10 DOI: 10.1007/s00393-024-01570-3
Johannes Knitza, Martin Krusche, Gamal Chehab, Christof Specker, Jutta G Richter

Digital health applications (DHAs) are revolutionising patient care by improving access to evidence-based therapy and promoting active self-management. The continuously growing number of DHAs enables patients to act more independently through digital support. The budget-neutral prescription and cost coverage by statutory health insurance companies reduce financial barriers for practitioners and patients. Initial studies show that DHAs can be used successfully to treat comorbidities and rheumatic diseases. Several DHAs for inflammatory rheumatic diseases are at an advanced stage of development. The identification of suitable patients and support through shared decision making are crucial for successful implementation. Challenges remain in adherence and acceptance of the applications. This article provides an overview of prescription in clinical routine, initial data and experiences from the reality of rheumatology care, and reports on current developments.

数字医疗应用程序(DHA)通过改善循证治疗的获取途径和促进积极的自我管理,正在彻底改变患者护理。数字健康应用程序的数量不断增加,使患者能够通过数字支持更加独立地行动。不影响预算的处方和由法定医疗保险公司承担的费用减少了从业人员和患者的经济障碍。初步研究表明,DHA 可成功用于治疗合并症和风湿性疾病。几种治疗炎症性风湿病的 DHA 已进入后期开发阶段。确定合适的患者并通过共同决策提供支持是成功实施的关键。在应用的依从性和接受度方面仍存在挑战。本文概述了临床常规处方、风湿病护理现实中的初步数据和经验,并报告了当前的发展情况。
{"title":"[Digital health applications-What we should know as rheumatologists].","authors":"Johannes Knitza, Martin Krusche, Gamal Chehab, Christof Specker, Jutta G Richter","doi":"10.1007/s00393-024-01570-3","DOIUrl":"https://doi.org/10.1007/s00393-024-01570-3","url":null,"abstract":"<p><p>Digital health applications (DHAs) are revolutionising patient care by improving access to evidence-based therapy and promoting active self-management. The continuously growing number of DHAs enables patients to act more independently through digital support. The budget-neutral prescription and cost coverage by statutory health insurance companies reduce financial barriers for practitioners and patients. Initial studies show that DHAs can be used successfully to treat comorbidities and rheumatic diseases. Several DHAs for inflammatory rheumatic diseases are at an advanced stage of development. The identification of suitable patients and support through shared decision making are crucial for successful implementation. Challenges remain in adherence and acceptance of the applications. This article provides an overview of prescription in clinical routine, initial data and experiences from the reality of rheumatology care, and reports on current developments.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2024-09-10","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142296727","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Nachruf auf Herrn Professor Dr. sc. med. Wolfgang Keitel. 沃尔夫冈-凯特尔教授的讣告。
IF 0.9 4区 医学 Q4 RHEUMATOLOGY Pub Date : 2024-09-03 DOI: 10.1007/s00393-024-01561-4
Eugen Feist, Karsten Beyer, Carlo Weimann
{"title":"Nachruf auf Herrn Professor Dr. sc. med. Wolfgang Keitel.","authors":"Eugen Feist, Karsten Beyer, Carlo Weimann","doi":"10.1007/s00393-024-01561-4","DOIUrl":"https://doi.org/10.1007/s00393-024-01561-4","url":null,"abstract":"","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":""},"PeriodicalIF":0.9,"publicationDate":"2024-09-03","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142126843","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Looking back on 51 years of the Carol Nachman Prize in Rheumatology-significance for the field of spondyloarthritis research. 回顾卡罗尔-纳赫曼风湿病学奖设立51年--对脊柱关节炎研究领域的意义。
IF 0.9 4区 医学 Q4 RHEUMATOLOGY Pub Date : 2024-09-01 Epub Date: 2024-06-12 DOI: 10.1007/s00393-024-01496-w
Jürgen Braun, Joachim Sieper, Elisabeth Märker-Hermann

The city and casino of Wiesbaden, capital of the German state Hessen, have endowed the Carol Nachman Prize to promote research work in the field of rheumatology since 1972. The prize, endowed with 37,500 €, is the second highest medical award in Germany and serves to promote clinical, therapeutic, and experimental research work in the field of rheumatology. In June 2022, the 50-year anniversary was celebrated. In the symposium preceding the award ceremony, an overview was given on the significance of spondyloarthritis for the work of the awardees in the past 30 years. This overview has now been put together to inform the interested community of the work performed, including the opinion of the awardees regarding what they consider to be their most important contribution.

德国黑森州首府威斯巴登市和威斯巴登赌场自 1972 年以来一直为促进风湿病学领域的研究工作而设立卡罗尔-纳赫曼奖。该奖奖金额为 37500 欧元,是德国第二高医学奖,旨在促进风湿病学领域的临床、治疗和实验研究工作。2022 年 6 月,该奖项迎来了 50 周年纪念。在颁奖仪式前的研讨会上,对脊柱关节炎在过去 30 年中对获奖者工作的意义进行了概述。现在,我们将这一概述整理成文,向感兴趣的各界人士介绍已开展的工作,包括获奖者对他们认为自己最重要的贡献的看法。
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引用次数: 0
[Procedures of complementary medicine in rheumatology]. [风湿病学中的补充医学程序]。
IF 0.9 4区 医学 Q4 RHEUMATOLOGY Pub Date : 2024-09-01 Epub Date: 2024-06-27 DOI: 10.1007/s00393-024-01524-9
Gernot Keyßer, Inna Frohne, Olaf Schultz, Monika Reuß-Borst, Oliver Sander, Alexander Pfeil

Patients with diseases of the musculoskeletal system are confronted with a large quantity of treatment offers based on methods of complementary medicine. Despite a considerable number of publications on this topic, the scientific evidence is still poor. This article focuses on Ayurvedic medicine (AM), traditional Chinese medicine (TCM), mind-body medicine and homeopathy. These procedures have a longstanding tradition of practice and each claims to have its own theoretical concept; however, the application in the field of rheumatology can only be recommended either for specific entities or, in the case of homeopathy, not at all. In addition, this article summarizes the evidence for dietary recommendations, nutritional supplements and herbal medicine in rheumatology. The latter topics are frequently discussed in the popular press and are a much-debated issue between physicians and patients; however, clear-cut recommendations for the application on a scientific basis are the exception and mainly consist of the endorsement to adhere to the principles of a Mediterranean diet.

肌肉骨骼系统疾病患者面临着大量基于补充医学方法的治疗方案。尽管有关这一主题的出版物数量可观,但科学证据仍然贫乏。本文重点介绍阿育吠陀医学(AM)、传统中医(TCM)、身心医学和顺势疗法。这些疗法有着悠久的实践传统,每种疗法都声称有自己的理论概念;然而,在风湿病学领域的应用只能针对特定的实体进行推荐,或者就顺势疗法而言,根本无法推荐。此外,本文还总结了风湿病学中饮食建议、营养补充剂和草药的证据。这些话题经常在大众媒体上讨论,也是医生和患者之间争论不休的问题;然而,有科学依据的明确应用建议是个例外,主要包括赞同坚持地中海饮食原则。
{"title":"[Procedures of complementary medicine in rheumatology].","authors":"Gernot Keyßer, Inna Frohne, Olaf Schultz, Monika Reuß-Borst, Oliver Sander, Alexander Pfeil","doi":"10.1007/s00393-024-01524-9","DOIUrl":"10.1007/s00393-024-01524-9","url":null,"abstract":"<p><p>Patients with diseases of the musculoskeletal system are confronted with a large quantity of treatment offers based on methods of complementary medicine. Despite a considerable number of publications on this topic, the scientific evidence is still poor. This article focuses on Ayurvedic medicine (AM), traditional Chinese medicine (TCM), mind-body medicine and homeopathy. These procedures have a longstanding tradition of practice and each claims to have its own theoretical concept; however, the application in the field of rheumatology can only be recommended either for specific entities or, in the case of homeopathy, not at all. In addition, this article summarizes the evidence for dietary recommendations, nutritional supplements and herbal medicine in rheumatology. The latter topics are frequently discussed in the popular press and are a much-debated issue between physicians and patients; however, clear-cut recommendations for the application on a scientific basis are the exception and mainly consist of the endorsement to adhere to the principles of a Mediterranean diet.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"549-561"},"PeriodicalIF":0.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141459626","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
[Aphthous ulcers in a patient of Turkish descent : A diagnostic challenge]. [土耳其裔患者的阿弗他溃疡:诊断难题]。
IF 0.9 4区 医学 Q4 RHEUMATOLOGY Pub Date : 2024-09-01 Epub Date: 2024-03-18 DOI: 10.1007/s00393-024-01498-8
Kathrin Standfest, Vanessa Bartsch, Manuela Beckert, Axel J Hueber
{"title":"[Aphthous ulcers in a patient of Turkish descent : A diagnostic challenge].","authors":"Kathrin Standfest, Vanessa Bartsch, Manuela Beckert, Axel J Hueber","doi":"10.1007/s00393-024-01498-8","DOIUrl":"10.1007/s00393-024-01498-8","url":null,"abstract":"","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"575-577"},"PeriodicalIF":0.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140159143","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
Anakinra in idiopathic recurrent pericarditis: a comprehensive case series and literature review. 用于特发性复发性心包炎的 Anakinra:综合病例系列和文献综述。
IF 0.9 4区 医学 Q4 RHEUMATOLOGY Pub Date : 2024-09-01 Epub Date: 2024-01-26 DOI: 10.1007/s00393-023-01471-x
Zeynep Toker Dincer, Sejla Karup, Erkin Yilmaz, Osman Corbali, Feyza Nur Azman, Melike Melikoglu, Serdal Ugurlu

Objective: Idiopathic recurrent pericarditis (IRP) is defined by recurring episodes of pericardial inflammation without a known cause. This study investigates the safety and efficacy of anakinra, an interleukin‑1 inhibitor, as a successful therapy for IRP in cases resistant to conventional treatment.

Methods: A retrospective evaluation of patients treated at our autoinflammatory center between 2011 and 2023 was conducted. Patient files were examined for demographic, clinical, and treatment response data, including nonsteroid anti-inflammatory drugs (NSAIDs), corticosteroids, and colchicine. Monogenic autoinflammatory disease screening was performed for Mediterranean Fever (MEFV), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), mevalonate kinase (MVK), nucleotide-binding domain, leucine-rich-containing family, pyrin domain-containing-3 (NLRP3), and nucleotide-binding oligomerization domain-containing protein 2 (NOD2). Patients who experienced multiple episodes of pericarditis were diagnosed with recurrent pericarditis. The study evaluated anakinra treatment in IRP patients unresponsive to conventional therapy.

Results: The study included 21 participants, 9 (42.9%) female and 12 (57.1%) male. The average age of the participants was 43.1 ± 16.5 years. The MEFV mutation analysis revealed that 2 (9.5%) had a mutation in exon 10 and 4 (19.0%) had one in exon 2. Out of the 16 cases, 15 successfully discontinued steroid treatment. Four patients (19.0%) experienced injection site reactions. C‑reactive protein (CRP) levels were measured at an average of 196 ± 67.8 mg/l before and 2.6 ± 3.15 mg/l after anakinra treatment.

Conclusion: In conclusion, the study adds to the growing evidence for the efficacy of interleukin-1 inhibitors, such as anakinra, as a promising treatment modality for IRP in cases resistant to conventional treatment.

目的:特发性复发性心包炎(IRP)是指无明确病因而反复发作的心包炎症。本研究探讨了白细胞介素-1抑制剂阿纳金拉(anakinra)的安全性和有效性,并将其作为一种成功的IRP治疗方法,用于常规治疗无效的病例:方法:对2011年至2023年期间在我院自体免疫炎症中心接受治疗的患者进行回顾性评估。检查了患者档案中的人口统计学、临床和治疗反应数据,包括非甾体抗炎药(NSAIDs)、皮质类固醇激素和秋水仙碱。对地中海热(MEFV)、肿瘤坏死因子受体相关周期性综合征(TRAPS)、甲羟戊酸激酶(MVK)、核苷酸结合域、富含亮氨酸家族、含吡啶结构域-3(NLRP3)和含核苷酸结合寡聚化结构域蛋白 2(NOD2)等单基因自身炎症性疾病进行了筛查。多次发作心包炎的患者被诊断为复发性心包炎。该研究评估了对常规疗法无反应的IRP患者的anakinra治疗效果:研究共纳入 21 名参与者,其中女性 9 人(42.9%),男性 12 人(57.1%)。参与者的平均年龄为(43.1 ± 16.5)岁。MEFV突变分析显示,2例(9.5%)在第10外显子发生突变,4例(19.0%)在第2外显子发生突变。在 16 例患者中,有 15 例成功中止了类固醇治疗。四名患者(19.0%)出现了注射部位反应。经测量,C反应蛋白(CRP)水平在阿纳金拉治疗前平均为(196 ± 67.8)毫克/升,治疗后平均为(2.6 ± 3.15)毫克/升:总之,这项研究为白细胞介素-1抑制剂(如anakinra)的疗效提供了更多证据,对于常规治疗无效的IRP病例,anakinra是一种很有前景的治疗方式。
{"title":"Anakinra in idiopathic recurrent pericarditis: a comprehensive case series and literature review.","authors":"Zeynep Toker Dincer, Sejla Karup, Erkin Yilmaz, Osman Corbali, Feyza Nur Azman, Melike Melikoglu, Serdal Ugurlu","doi":"10.1007/s00393-023-01471-x","DOIUrl":"10.1007/s00393-023-01471-x","url":null,"abstract":"<p><strong>Objective: </strong>Idiopathic recurrent pericarditis (IRP) is defined by recurring episodes of pericardial inflammation without a known cause. This study investigates the safety and efficacy of anakinra, an interleukin‑1 inhibitor, as a successful therapy for IRP in cases resistant to conventional treatment.</p><p><strong>Methods: </strong>A retrospective evaluation of patients treated at our autoinflammatory center between 2011 and 2023 was conducted. Patient files were examined for demographic, clinical, and treatment response data, including nonsteroid anti-inflammatory drugs (NSAIDs), corticosteroids, and colchicine. Monogenic autoinflammatory disease screening was performed for Mediterranean Fever (MEFV), tumor necrosis factor receptor-associated periodic syndrome (TRAPS), mevalonate kinase (MVK), nucleotide-binding domain, leucine-rich-containing family, pyrin domain-containing-3 (NLRP3), and nucleotide-binding oligomerization domain-containing protein 2 (NOD2). Patients who experienced multiple episodes of pericarditis were diagnosed with recurrent pericarditis. The study evaluated anakinra treatment in IRP patients unresponsive to conventional therapy.</p><p><strong>Results: </strong>The study included 21 participants, 9 (42.9%) female and 12 (57.1%) male. The average age of the participants was 43.1 ± 16.5 years. The MEFV mutation analysis revealed that 2 (9.5%) had a mutation in exon 10 and 4 (19.0%) had one in exon 2. Out of the 16 cases, 15 successfully discontinued steroid treatment. Four patients (19.0%) experienced injection site reactions. C‑reactive protein (CRP) levels were measured at an average of 196 ± 67.8 mg/l before and 2.6 ± 3.15 mg/l after anakinra treatment.</p><p><strong>Conclusion: </strong>In conclusion, the study adds to the growing evidence for the efficacy of interleukin-1 inhibitors, such as anakinra, as a promising treatment modality for IRP in cases resistant to conventional treatment.</p>","PeriodicalId":23834,"journal":{"name":"Zeitschrift fur Rheumatologie","volume":" ","pages":"587-596"},"PeriodicalIF":0.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139564724","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
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Zeitschrift fur Rheumatologie
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