pericardial diseases

  • 文章类型: Journal Article
    背景:复发性心包炎(RP)是一种与高发病率相关的复杂疾病。先前的研究已经评估了哪些变量与临床缓解相关。然而,目前尚无已建立的风险分层模型来预测这些患者的结局.
    目的:我们开发了一个风险分层模型,该模型可以预测RP患者的长期结局,并能够识别具有预后不良特征的患者。
    方法:我们回顾性研究了2012年至2019年的365例RP患者。主要结果是临床缓解(CR),定义为停止所有抗炎治疗,症状完全缓解。使用五种机器学习生存模型来计算5年内CR的可能性,并将患者分层为高风险,中等风险,低风险人群。
    结果:在队列中,平均年龄为46±15岁,205人(56%)是女性。118例(32%)患者获得CR。最终的模型包括类固醇依赖性,复发的总数,心包晚钆增强,年龄,病因学,性别,射血分数,心率是最重要的参数。该模型在测试集上的C指数为0.800预测结果,并表现出将患者分层为低风险的显着能力。中等风险,和高危人群(对数秩检验;P<0.0001)。
    结论:我们开发了一种新的风险分层模型来预测RP患者的CR。我们的模型还可以帮助患者分层,具有较高的辨别能力。使用可解释的机器学习模型可以帮助医生在RP患者中做出个性化的治疗决策。
    BACKGROUND: Recurrent pericarditis (RP) is a complex condition associated with significant morbidity. Prior studies have evaluated which variables are associated with clinical remission. However, there is currently no established risk-stratification model for predicting outcomes in these patients.
    OBJECTIVE: We developed a risk stratification model that can predict long-term outcomes in patients with RP and enable identification of patients with characteristics that portend poor outcomes.
    METHODS: We retrospectively studied a total of 365 consecutive patients with RP from 2012 to 2019. The primary outcome was clinical remission (CR), defined as cessation of all anti-inflammatory therapy with complete resolution of symptoms. Five machine learning survival models were used to calculate the likelihood of CR within 5 years and stratify patients into high-risk, intermediate-risk, and low-risk groups.
    RESULTS: Among the cohort, the mean age was 46 ± 15 years, and 205 (56%) were women. CR was achieved in 118 (32%) patients. The final model included steroid dependency, total number of recurrences, pericardial late gadolinium enhancement, age, etiology, sex, ejection fraction, and heart rate as the most important parameters. The model predicted the outcome with a C-index of 0.800 on the test set and exhibited a significant ability in stratification of patients into low-risk, intermediate-risk, and high-risk groups (log-rank test; P < 0.0001).
    CONCLUSIONS: We developed a novel risk-stratification model for predicting CR in RP. Our model can also aid in stratifying patients, with high discriminative ability. The use of an explainable machine learning model can aid physicians in making individualized treatment decision in RP patients.
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  • 文章类型: Case Reports
    我们介绍了一例有趣的分枝杆菌结核心包炎病例,该病例表现为海地裔年轻的墨西哥移民早期心脏压塞的渗出性缩窄性心包炎。患者接受了心包窗,并接受了利福平治疗,异烟肼,吡嗪酰胺,乙胺丁醇,和维生素B6。在进一步接受类固醇治疗后,病人情况良好,安全出院回家。
    We present an interesting case of mycobacterial tuberculosis pericarditis presenting as effusive constrictive pericarditis with early cardiac tamponade in a young Mexican migrant of Haitian descent. The patient underwent a pericardial window and was treated with rifampin, isoniazid, pyrazinamide, ethambutol, and vitamin B6. After further receiving steroids, the patient was doing well and was discharged home safely.
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  • 文章类型: Journal Article
    背景心包疾病表现为各种临床形式,包括急性心包炎,缩窄性心包炎,心包积液,和心脏填塞,以急性心包炎最为普遍.这些情况极大地导致了死亡率。因此,本文旨在根据年龄和性别分析巴西人口的死亡率趋势,阐明心包疾病对公共卫生结果的影响。方法对巴西(2000-2022年)心包疾病死亡率进行回顾性时间序列分析。数据来自统一卫生系统(DATASUS)的信息学部门,并纳入第10版国际疾病分类(ICD-10)编码:I30,I31和I32进行分析.我们从巴西地理与统计研究所(IBGE)收集了按年龄范围和性别分类的人口和人口统计数据。随后,我们计算了每100,000个人的年龄标准化死亡率,并使用连接点回归评估了年度百分比变化(APC)和平均年度百分比变化(AAPC),以及它们相应的95%置信区间(CI)。结果在基于性别的死亡率趋势方面,在研究期间,男性和合并性别的总死亡率保持稳定.然而,女性死亡率显著上升(AAPC=1.18),特别是在2020年至2022年之间,APC为27.55。分析不同年龄段(20至80岁及以上)的心包疾病,观察到在整个研究期间,70-79岁和80岁及以上年龄组的死亡率显著增加(分别为AAPC=1.0339和AAPC=3.4587).这两个年龄组在2020年至2022年期间的死亡率增幅最高。其他年龄组的AAPC没有显着变化。结论这项跨越二十年(2000-2022年)的综合分析,研究了巴西心包疾病的死亡率趋势,并揭示了总体相对稳定。男性由于心包疾病而表现出总体较高的死亡率;然而,女性在整个时期的死亡率上升趋势最为显著。在第一部分(2000-2015)中,死亡率在所有队列中上升,这归因于不合格的医疗设施和结核病等传染病。第二部分(2016-2020年)死亡率下降,可能是由于医疗保健的改善,特别是超声心动图的可用性增加。然而,第三部分(2020-2022)见证了死亡率的急剧上升,恰逢COVID-19大流行,有COVID-19后症状,尤其是心包炎.与心包积液相比,心包炎相关的死亡率下降,死亡率与年龄直接相关,由于合并症增加,老年人群的死亡率更高,健康和免疫能力下降。
    Background Pericardial diseases manifest in various clinical forms, including acute pericarditis, constrictive pericarditis, pericardial effusion, and cardiac tamponade, with acute pericarditis being the most prevalent. These conditions significantly contribute to mortality rates. Therefore, this article aimed to analyze mortality trends in the Brazilian population based on age and sex, shedding light on the impact of pericardial diseases on public health outcomes. Methods  This is a retrospective time-series analysis of pericardial disease mortality rates in Brazil (2000-2022). Data was obtained from the Department of Informatics of the Unified Health System (DATASUS), and the 10th edition of the International Classification of Diseases (ICD-10) codes: I30, I31, and I32 were included for analysis. We gathered population and demographic data categorized by age range and sex from the Brazilian Institute of Geography and Statistics (IBGE). Subsequently, we computed the age-standardized mortality rate per 100,000 individuals and assessed the annual percentage changes (APCs) and average annual percentage changes (AAPCs) using joinpoint regression, along with their corresponding 95% confidence intervals (CIs). Results  In terms of mortality trends based on sex, overall mortality rates remained stable for males and combined sexes over the study period. However, there was a notable increase in mortality rates among females (AAPC=1.18), particularly between 2020 and 2022, with a significant APC of 27.55. Analyzing pericardial diseases across different age groups (20 to 80 years and above), it wasobserved that mortality rates significantly increased in the 70-79 and 80 years and above age groups throughout the study period (AAPC=1.0339 and AAPC=3.4587, respectively). These two age groups experienced the highest significant rise in mortality between 2020 and 2022. Other age groups did not exhibit a significant change in AAPC. Conclusions  This comprehensive analysis spanning two decades (2000-2022), examined the mortality trends of pericardial diseases in Brazil and revealed relative stability overall. Males exhibited an overall higher mortality number due to pericardial diseases; however, females showed the most significant increase in mortality trend throughout the whole period. In the first segment (2000-2015), mortality rose across all cohorts, which was attributed to substandard healthcare facilities and infectious diseases like tuberculosis. The second segment (2016-2020) saw a decline in mortality, likely due to improved healthcare, particularly the increased availability of echocardiograms. However, the third segment (2020-2022) witnessed a sharp rise in mortality, coinciding with the COVID-19 pandemic, with post-COVID-19 symptoms, particularly pericarditis. Pericarditis-related death rates declined compared to pericardial effusion, and mortality rates correlated directly with age, with older cohorts experiencing higher mortality due to increased comorbidities, and decline in health and immunocompetency.
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  • 文章类型: Journal Article
    背景胸部放疗是一种广泛使用的胸部肿瘤治疗和姑息性治疗选择。然而,辐射暴露的短期和长期心血管不良反应仍然是一个主要问题.短期不良反应在暴露后数月内观察到,如心包疾病;同时,长期并发症通常是阴险的,几十年来明显的,比如充血性心力衰竭,冠状动脉疾病,心肌病,传导障碍,缩窄性心包炎,和心脏瓣膜病.因此,长期的心血管不良反应很难预测,与辐射暴露的关联仍然难以建立。方法本回顾性研究,观察性研究使用2016年至2019年国家住院患者样本(NIS)数据库的数据进行.使用主要和次要国际疾病分类定义接受放射治疗(RT)的原发性胸部恶性肿瘤的成年患者,第十修订代码。可以用RT治疗的其他恶性肿瘤和所有继发性恶性肿瘤从主要对照组中排除。心脏结果定义为充血性心力衰竭的患病率,冠状动脉疾病,心肌病,传导障碍,心包疾病,和原发性心脏瓣膜病。多变量逻辑和线性回归分析用于校正混杂因素。结果与普通人群相比,暴露于RT的胸部恶性肿瘤的成年人患慢性心包炎的几率较高(校正比值比(aOR)=2,95%置信区间(CI)=1.9-2.2,p<0.001),急性心包炎(aOR=2.3,95%CI=1.9-2.9,p<0.001),缩窄性心包炎(aOR=2.8,95%CI=2.1-3.7,p<0.001),传导障碍(aOR=1.3,95%CI=1.2-1.35,p<0.001),冠状动脉疾病(aOR=1.24,95%CI=1.2-1.27,p<0.001),心力衰竭(aOR=1.44,95%CI=1.4-1.5,p<0.001),和心脏瓣膜病(aOR=1.37,95%CI=1.3-1.4,p<0.001)。发生心脏骤停(aOR=1,95%CI=0.9-1.10,p=0.6)或急性心肌梗死(aOR=1.1,95%CI=1-1.15,p<0.001)的几率没有差异。与未暴露于RT的患有胸部恶性肿瘤的成年人相比,暴露于RT的患有胸部恶性肿瘤的成年人发生急性心肌梗死的几率更高(aOR=1.14,95%CI=1.1-1.18,p<0.001),慢性心包炎(aOR=1.3,95%CI=1.2-1.3,p<0.001),急性心包炎(aOR=1.6,95%CI=1.2-2.1,p<0.001),缩窄性心包炎(aOR=2.2,95%CI=1.5-3.2,p<0.001),传导障碍(aOR=1.1,95%CI=1.08-1.13,p<0.001),冠状动脉疾病(aOR=1.14,95%CI=1.12-1.16,p<0.001),心力衰竭(aOR=1.2,95%CI=1.17-1.23,p<0.001),和瓣膜性心脏病(aOR=1.3,95%CI=1.2-1.35,p<0.001)。两组发生心脏骤停的几率相似(aOR=0.86,95%CI=0.8-0.98,p=0.05)。结论接受RT治疗的患有胸部恶性肿瘤的成年人患慢性心包炎的几率更高,急性心包炎,缩窄性心包炎,传导障碍,冠状动脉疾病,心力衰竭,与普通成年人相比,心脏瓣膜病和心脏瓣膜病发生心脏骤停或急性心肌梗死的几率相似。
    Background Thoracic irradiation is a widely used therapeutic and palliative treatment option for thoracic neoplasms. However, short- and long-term cardiovascular adverse effects of radiation exposure remain a major concern. The short-term adverse effects are observed within months of exposure such as pericardial diseases; meanwhile, the long-term complications are usually insidious and manifest over decades, such as congestive heart failure, coronary artery disease, cardiomyopathy, conduction disorders, constrictive pericarditis, and valvular heart disease. Hence, long-term cardiovascular adverse effects are challenging to predict, and the association with radiation exposure remains difficult to establish. Methodology This retrospective, observational study was conducted using data from the National Inpatient Sample (NIS) database from 2016 to 2019. Adult patients with primary thoracic malignancies who underwent radiation therapy (RT) were defined using principal and secondary International Classification of Diseases, Tenth Revision codes. Other malignancies that can be treated with RT and all secondary malignancies were excluded from the primary comparison group. Cardiac outcomes were defined as the prevalence of congestive heart failure, coronary artery disease, cardiomyopathy, conduction disorders, pericardial diseases, and valvular heart diseases in the primary group. The multivariate logistic and the linear regression analyses were used to adjust for confounders. Results When compared to the general population, adults with thoracic malignancies exposed to RT had higher odds of developing chronic pericarditis (adjusted odds ratio (aOR) = 2, 95% confidence interval (CI) = 1.9-2.2, p < 0.001), acute pericarditis (aOR = 2.3, 95% CI = 1.9-2.9, p < 0.001), constrictive pericarditis (aOR = 2.8, 95% CI = 2.1-3.7, p < 0.001), conduction disorders (aOR = 1.3, 95% CI = 1.2-1.35, p < 0.001), coronary artery disease (aOR = 1.24, 95% CI = 1.2-1.27, p < 0.001), heart failure (aOR = 1.44, 95% CI = 1.4-1.5, p < 0.001), and valvular heart disease (aOR = 1.37, 95% CI = 1.3-1.4, p < 0.001). There was no difference in the odds of developing cardiac arrest (aOR = 1, 95% CI = 0.9-1.10, p = 0.6) or acute myocardial infarction (aOR = 1.1, 95% CI = 1-1.15, p < 0.001). When compared to adults with thoracic malignancies not exposed to RT, adults with thoracic malignancies who were exposed to RT had higher odds of developing acute myocardial infarction (aOR = 1.14, 95% CI = 1.1-1.18, p < 0.001), chronic pericarditis (aOR = 1.3, 95% CI = 1.2-1.3, p < 0.001), acute pericarditis (aOR = 1.6, 95% CI = 1.2-2.1, p < 0.001), constrictive pericarditis (aOR = 2.2, 95% CI = 1.5-3.2, p < 0.001), conduction disorders (aOR = 1.1, 95% CI = 1.08-1.13, p < 0.001), coronary artery disease (aOR = 1.14, 95% CI = 1.12-1.16, p < 0.001), heart failure (aOR = 1.2, 95% CI = 1.17-1.23, p < 0.001), and valvular heart disease (aOR = 1.3, 95% CI = 1.2-1.35, p < 0.001). The odds were similar between the two groups for developing cardiac arrest (aOR = 0.86, 95% CI = 0.8-0.98, p = 0.05). Conclusions Adults with thoracic malignancies who were treated with RT have higher odds of developing chronic pericarditis, acute pericarditis, constrictive pericarditis, conduction disorders, coronary artery disease, heart failure, and valvular heart disease while similar odds of developing cardiac arrest or acute myocardial infarction compared to the general adult population.
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  • 文章类型: Journal Article
    心脏磁共振(CMR)成像通过提供心脏解剖和功能的信息,已被确立为评估心包疾病的有价值的诊断工具。围绕着心脏外结构,心包增厚和积液,心包积液的表征,以及来自同一扫描的活动性心包炎症的存在。此外,CMR成像对于收缩生理的非侵入性检测具有出色的诊断准确性,在大多数情况下避免了对侵入性导管插入的需要。该领域越来越多的证据表明,CMR的心包增强不仅可以诊断心包炎,而且对心包炎复发具有预后价值。尽管这些证据来自小型患者队列。CMR的发现也可用于指导复发性心包炎的治疗降阶梯或滴定,并选择最有可能从新治疗中受益的患者,例如anakinra和rilonacept。本文概述了CMR在心包综合征中的应用,作为报告医生的入门。我们试图提供所使用的临床方案的摘要以及对心包疾病中主要CMR发现的解释。我们还讨论了一些不太清楚的观点,并描述了CMR在心包疾病中的优缺点。
    Cardiac magnetic resonance (CMR) imaging has been established as a valuable diagnostic tool in the assessment of pericardial diseases by providing information on cardiac anatomy and function, surrounding extra-cardiac structures, pericardial thickening and effusion, characterization of pericardial effusion, and the presence of active pericardial inflammation from the same scan. In addition, CMR imaging has excellent diagnostic accuracy for the non-invasive detection of constrictive physiology evading the need for invasive catheterization in most instances. Growing evidence in the field suggests that pericardial enhancement on CMR is not only diagnostic of pericarditis but also has prognostic value for pericarditis recurrence, although such evidence is derived from small patient cohorts. CMR findings could also be used to guide treatment de-escalation or up-titration in recurrent pericarditis and selecting patients most likely to benefit from novel treatments such as anakinra and rilonacept. This article is an overview of the CMR applications in pericardial syndromes as a primer for reporting physicians. We sought to provide a summary of the clinical protocols used and an interpretation of the major CMR findings in the setting of pericardial diseases. We also discuss points that are less well clear and delineate the strengths and weak points of CMR in pericardial diseases.
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  • 文章类型: Journal Article
    心肌炎是COVID-19疫苗报道的并发症之一,特别是辉瑞生物技术公司和Moderna疫苗。有关此关联的大多数已发布数据来自病例报告和系列。整合地理数据,临床表现,因此,对于心肌炎患者,结局对更好地了解该病非常重要.在Cochrane图书馆进行了彻底的文献检索,PubMed,ScienceDirect,以及截至2022年3月30日发表文献的谷歌学者。我们从29项研究中确定了26例符合条件的患者;数据来自这些符合条件的病例报告和病例系列。在这项研究中,大约94%的患者是男性,心肌炎的中位发病年龄为22岁,85%的患者在第二次给药后出现症状.接种疫苗后患者入院的中位时间为三天,胸痛是这些患者中最常见的症状。大多数患者入院时肌钙蛋白升高,约90%的患者进行了心脏磁共振成像(CMR),显示钆增强。所有入院的心肌炎患者在中位住院四天后出院。当前分析的结果表明,mRNA疫苗接种后心肌炎主要见于第二次疫苗接种后的年轻男性。疫苗诱导的心肌炎的病理生理学尚不完全清楚,在这些患者中,CMR的晚期钆增强是常见的发现,可能表明心肌纤维化或坏死。预后仍然良好,所有患者都从心肌炎中康复,然而,建议进一步研究以评估心肌炎的长期预后.
    Myocarditis is one of the complications reported with COVID-19 vaccines, particularly both Pfizer-BioNTech and Moderna vaccines. Most of the published data about this association come from case reports and series. Integrating the geographical data, clinical manifestations, and outcomes is therefore important in patients with myocarditis to better understand the disease. A thorough literature search was conducted in Cochrane library, PubMed, ScienceDirect, and Google Scholar for published literature till 30 March 2022. We identified 26 patients eligible from 29 studies; the data were pooled from these qualifying case reports and case series. Around 94% of patients were male in this study, the median age for onset of myocarditis was 22 years and 85% developed symptoms after the second dose. The median time of admission for patients to hospitals post-vaccination was three days and chest pain was the most common presenting symptom in these patients. Most patients had elevated troponin on admission and about 90% of patients had cardiac magnetic resonance imaging (CMR) that showed late gadolinium enhancement. All patients admitted with myocarditis were discharged home after a median stay of four days. Results from this current analysis show that post-mRNA vaccination myocarditis is mainly seen in young males after the second dose of vaccination. The pathophysiology of vaccine-induced myocarditis is not entirely clear and late gadolinium enhancement is a common finding on CMR in these patients that may indicate myocardial fibrosis or necrosis. Prognosis remains good and all patients recovered from myocarditis, however further studies are advisable to assess long-term prognosis of myocarditis.
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  • 文章类型: Journal Article
    BACKGROUND: Acute pericarditis has a wide spectrum of clinical presentations largely depending on underlying aetiologies. We assessed the role of age and sex in the clinical features and outcome of acute pericarditis.
    METHODS: A total of 240 consecutive patients hospitalized with a first episode of acute pericarditis were included. At baseline demographics, clinical features, laboratory and imaging findings and medical therapy were recorded. Patients were followed up for at least 18 months for complications. Data comparisons were performed according to sex and age (≤60 or >60 years).
    RESULTS: The male/female ratio was 1.42, and 56% of patients were >60 years. Younger patients depicted more often chest pain (P = .001), fever and rubs (P < .001 for both), ST elevation and PR depression (P = .032 and .009, respectively), higher CRP values (P = .009) and less often dyspnoea (P = .046) and pericardial effusion (P = .036). Moreover, they received less often glucocorticoids (P < .001) and depicted less atrial fibrillation (P = .003) and a higher rate of recurrent pericarditis (P = .013). After multivariate adjustment for confounders, age >60 years remained an independent predictor for a lower risk of recurrent pericarditis (hazard ratio 0.60, 95% CI: 0.39-0.96, P = .033). Regarding sex, females were older (P = .007), showed less often ST elevation and PR depression (P < .001 and .002, respectively) and had a higher baseline heart rate (P = .02). Sex was not associated with recurrent pericarditis risk.
    CONCLUSIONS: Patients with acute pericarditis have distinct presenting clinical, biochemical and prognostic features according to age and sex. Awareness of such differences is important for clinical decision-making.
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  • 文章类型: Journal Article
    Background Pericardial effusion is the accumulation of blood or excess fluid in the cavity between the heart and the pericardium sac. Pericardial effusion can be caused by several etiologies, including malignant and non-malignant causes. Pericardiocentesis is the gold standard assessment method for pericardial effusion etiology. The aim of this study was to identify the long-term outcome of patients who presented with massive pericardial effusion and underwent pericardiocentesis at King Abdulaziz Medical City, Jeddah, a large tertiary hospital in the western part of Saudi Arabia. Methods This is a single-center retrospective cross-sectional study conducted at King Abdulaziz Medical City Jeddah, Saudi Arabia, between January 2013 to December 2018. Data were collected from patient\'s charts; the clinical and echocardiographic findings, alongside with pericardial fluid analysis, were collected. Procedure and patients outcomes were obtained and reported. Results Of the 107 patients with pericardial effusion, 39 patients had moderate to severe pericardial effusion requiring pericardiocentesis. The mean age was 52 years, and 56.4% were females. The most common chronic disease was hypertension and the presence of metastasis. The most common cause of pericardial effusion was a malignancy. A majority of patients had severe pericardial effusion. Many patients had tamponade (69.6%). Patients with malignant pericardial effusion had a median survival of 54 days. Conclusion Etiologies of pericardial effusion requiring drainage depend on the population studied. Patients with malignant effusions have worse outcomes than non-malignant effusion. Pericardiocentesis is required to ascertain the cause and risk-stratify patients.
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  • 文章类型: Journal Article
    This CME review takes stock of the progress in the etiology, pathophysiology, diagnostics and treatment of pericarditis and pericardial effusion brought about by the publication of the 2nd European Society of Cardiology (ESC) guidelines on the management of pericardial diseases in 2015. It also emphasizes special forms, which have received less attention in the past, such as therapy-refractory (incessant), effusive-constrictive and constrictive pericarditis and the treatment of acute and recurrent pericarditis with colchicine. After the diagnosis of pericarditis with or without effusion has been made, the first step is to clarify its etiology, which affects the clinical symptoms, course, treatment and the prognosis. In this aspect the requirements of the guidelines and the reality of an etiological classification of pericardial diseases diverge in many cases. The diagnosis of \"idiopathic\" acute or recurrent pericarditis is still much too often the result of insufficient efforts to find the cause. Too often only malignant and bacterial forms are excluded. If the etiology is known local intrapericardial treatment with the already inserted pigtail catheter from the diagnostic pericardial puncture can be carried out with few systemic side effects. The 2015 ESC guidelines recommend colchicine as first line treatment in all forms of pericarditis except for neoplastic pericardial effusion. It accelerates healing and reduces the frequency of recurrence of pericarditis but cannot eliminate recurrence completely. The best treatment and prevention of recurrence is the eradication of the underlying etiological cause.
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  • 文章类型: Journal Article
    Recurrent pericarditis is one of the most frequent pericardial diseases, affecting up to 30% of the patients who have experienced acute pericarditis. While the diagnosis of acute pericarditis is sometime straight forward, its etiology and therapeutic management are still a challenge for physicians. In developed countries, the idiopathic form is the most frequent, and the search for an infectious etiology is almost invariably negative. Nevertheless, since standard treatment with nonsteroidal anti-inflammatory drugs and colchicine is not always able to neutralize pericardial inflammation in recurrent pericarditis, anakinra, an IL-1 receptor antagonist, has been proposed as a possible therapeutic alternative for refractory forms. IL-1 is a cytokine that exerts a pivotal role in innate immunity and in the pathogenesis of some autoimmune diseases, such as rheumatoid arthritis, and in autoinflammatory disorders, as familial Mediterranean fever and cryopyrin-associated periodic syndromes. The successful management of patients with acute idiopathic recurrent pericarditis (IRP) needs a teamwork approach, where cardiologists, rheumatologists, clinical immunologists and internists are involved. In this review, we will discuss the clinical and therapeutical challenges of IRP both in adults and children from a clinical practice standpoint. We will also briefly illustrate the main pathogenic mechanisms of IRP to provide internists and cardiologists with the rationale for approaching the use of anakinra in selected clinical cases.
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