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69 Atypical ataxia: a rare case of mycoplasma pneumonia meningoencephalitis
IF 2.3 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-02-01 DOI: 10.1016/S0002-9629(25)00078-3
AL Seidenburg, K Thetford
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引用次数: 0
CT-based body composition parameters predict the loss of response to infliximab in patients with Crohn's disease 基于 CT 的身体成分参数可预测克罗恩病患者对英夫利西单抗失去反应。
IF 2.3 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-02-01 DOI: 10.1016/j.amjms.2024.08.025
Shaotang Li MD , Hao Wu MB , Shouliang Miao MSc , Chen Huang MB , Yini Zhang MSc , Xinyi Shao MSc , Chao Chen MSc , Xiaoli Wu MD

Objective

Infliximab is a first-line biologic agent for the treatment of Crohn's disease (CD), in which loss of response (LOR) remains a challenge in the treatment of patients with CD. The study aimed to explore the association between body composition parameters and LOR to infliximab in CD patients.

Methods

118 patients with CD admitted to the First Affiliated Hospital of Wenzhou Medical University and treated with infliximab from June 2015 to December 2021 were retrospectively enrolled. The body composition of patients was analyzed by computed tomography (CT). The primary outcome measure was the one-year LOR. Patients were divided into the Remission group and the LOR group to analyze the association between body composition parameters and the LOR to infliximab.

Results

The rate of sarcopenia in the LOR group was higher than in the Remission group (83.7% vs. 60.0%, P=0.008). Multivariate analysis showed that females had a lower risk of sarcopenia than males (OR=0.30, 95% CI 0.11-0.81, P =0.017); BMI was significantly associated with sarcopenia (OR=0.68, 95% CI 0.56-0.83, P <0.001); L1 CD and L2 CD had a lower risk of sarcopenia than L3 CD (OR=0.29, 95% CI 0.10-0.83, P =0.021; OR=0.25, 95% CI 0.07-0.87, P=0.028).

Conclusions

Sarcopenia was identified as a risk factor for developing LOR in infliximab-treated patients.
目的:英夫利昔单抗是治疗克罗恩病(CD)的一线生物制剂:英夫利昔单抗是治疗克罗恩病(Crohn's disease,CD)的一线生物制剂。该研究旨在探讨身体成分参数与英夫利昔单抗治疗CD患者LOR之间的关系。方法:回顾性入选温州医科大学附属第一医院2015年6月至2021年12月收治的118例接受英夫利昔单抗治疗的CD患者。通过计算机断层扫描(CT)分析了患者的身体成分。主要结局指标为一年的LOR。患者被分为缓解组和LOR组,以分析身体成分参数与英夫利西单抗LOR之间的关系:结果:LOR组的肌少症发生率高于缓解组(83.7%对60.0%,P=0.008)。多变量分析显示,女性患肌肉疏松症的风险低于男性(OR=0.30,95%CI 0.11-0.81,P=0.017);体重指数与肌肉疏松症显著相关(OR=0.68,95%CI 0.56-0.83,P 结论:肌肉疏松症被认为是一种严重的慢性疾病:肌肉疏松症是英夫利西单抗治疗患者发生LOR的一个风险因素。
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引用次数: 0
35 Aplasia cutis congenita following radiofrequency ablative reduction of multiple fetuses in a monochorionic triamniotic pregnancy
IF 2.3 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-02-01 DOI: 10.1016/S0002-9629(25)00044-8
KN Gurukkal
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引用次数: 0
49 Goltz syndrome in a neonate
IF 2.3 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-02-01 DOI: 10.1016/S0002-9629(25)00058-8
NP Callahan, P Lertwilaiwittaya, JB Philips
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引用次数: 0
41 Endocarditis in a neonate after patent ductus arteriosus occluder placement
IF 2.3 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-02-01 DOI: 10.1016/S0002-9629(25)00050-3
K Fagundes, M Murphy, A Muralidhar, D Palazzi, MH Premkumar, D Dinu
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引用次数: 0
Elevated triglyceride-glucose index as a predictor of carotid plaque incidence: Insights from a comprehensive meta-analysis 作为颈动脉斑块发病率预测因子的甘油三酯-葡萄糖指数升高:综合元分析的启示。
IF 2.3 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-02-01 DOI: 10.1016/j.amjms.2024.08.013
Arankesh Mahadevan MBBS , Bhavin A. Patel MD , Sashwath Srikanth MD , Raja Godasi MBBS , Rupak Desai MBBS

Background

Early detection of carotid plaque (CP) can help reduce the burden of ASCVD in the general population. CP and triglyceride-glucose index (TyGi) are associated with insulin resistance.

Objectives

We performed a meta-analysis exploring the association of TyGi levels with the incidence of CP.

Methods

A systematic search of PubMed, Scopus, and Google Scholar till September 2023 reporting TyGi and CP identified 67 studies, of which 13 met our study criteria. TyGi was assessed both categorically and continuously. Binary random-effects models used for pooled odds ratios (OR) with 95% confidence intervals (CI) and I2 statistic for heterogeneity.

Results

Analyzed data from 163,792 patients with a mean age of 53 ± 8.9 years, primarily female (51.5%). Common comorbidities were hypertension (42.4%) followed by dyslipidemia (24.3%). Multivariable analysis showed that high vs. low TyGi quartile was associated with a higher risk of CP with unadjusted OR (1.82, 95% CI [1.5 - 2.21], p < 0.01; I² = 95.77, p < 0.01) and adjusted OR (1.3, 95% CI [1.16 - 1.46], p < 0.01; I² = 79.71, p < 0.01). Increasing TyGi also had a higher risk of CP with unadjusted OR (1.53, 95% CI [1.15 - 2.03], p < 0.01; I² = 98.48, p < 0.01) and adjusted OR (1.23, 95% CI [1.11 - 1.35], p < 0.01; I² = 89.82, p < 0.01). The association was validated by sensitivity analysis.

Conclusion

Our study indicates a higher risk of CP in patients with higher TyGi scores, underscoring its significance as a predictor for carotid atherosclerosis.
背景:颈动脉斑块(CP)的早期检测有助于减轻ASCVD在普通人群中的负担。CP和甘油三酯-葡萄糖指数(TyGi)与胰岛素抵抗有关:我们进行了一项荟萃分析,探讨了 TyGi 水平与冠状动脉粥样硬化发病率的关系:截至 2023 年 9 月,我们在 PubMed、Scopus 和 Google Scholar 上系统检索了 67 项报道 TyGi 和 CP 的研究,其中 13 项符合我们的研究标准。对TyGi进行了分类和连续评估。二元随机效应模型用于汇总赔率(OR)及95%置信区间(CI)和异质性I2统计:分析了 163 792 名患者的数据,他们的平均年龄为 53 ± 8.9 岁,主要为女性(51.5%)。常见合并症是高血压(42.4%),其次是血脂异常(24.3%)。多变量分析显示,TyGi四分位数高与低与CP风险较高相关,未调整OR(1.82,95% CI [1.5-2.21],p < 0.01;I² = 95.77,p < 0.01)和调整OR(1.3,95% CI [1.16-1.46],p < 0.01;I² = 79.71,p < 0.01)。TyGi越高,CP风险也越高,未调整OR(1.53,95% CI [1.15-2.03],p< 0.01;I² = 98.48,p< 0.01)和调整OR(1.23,95% CI [1.11-1.35],p< 0.01;I² = 89.82,p< 0.01)。这一关联通过敏感性分析得到了验证:我们的研究表明,TyGi评分越高的患者罹患CP的风险越高,这凸显了TyGi作为颈动脉粥样硬化预测因子的重要性。
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引用次数: 0
Single nucleotide polymorphisms of GEMIN3 modify the risk of primary Sjögren's syndrome in female patients GEMIN3 的单核苷酸多态性改变了女性患者罹患原发性斯约格伦综合征的风险。
IF 2.3 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-02-01 DOI: 10.1016/j.amjms.2024.07.001
Dong Wang PhD , Jingjing Zhang MD , Yufei Zhao MD , Ruijie Cao MD , Yingnan Wang MD , Iren Guo Student , Chenxing Peng PhD , Yanrong Song MD , Shasha Zhang MD

Background

MicroRNA (miRNA)-processing machinery may modify the risk of primary Sjögren's syndrome (pSS) by altering miRNA expression profiles. Inflammatory cytokines and reactive oxygen species (ROS) are also involved in pSS; however, the role of altered miRNAs expression in its pathogenesis is still unclear. We aimed to evaluate the relationship between single-nucleotide polymorphisms (SNPs) in miRNA processing machinery genes, including XPO5 (rs11077), RAN (rs14035), Dicer (rs3742330), TNRC6B (rs9623117), GEMIN3 (rs197412), and GEMIN4 (rs2740348), and the risk of pSS in female patients. The potential associations of cytokines and ROS with pSS-susceptible SNPs were also evaluated.

Materials and Methods

The SNPs confirmed by polymerase chain reaction ligase detection reaction were genotyped in 74 female patients with pSS and 77 controls. The relationship was analyzed by Student's t-test, Wilcoxon rank-sum test, chi-square test, Pearson's correlation test, and binary logistic regression analysis.

Results

For rs197412 of the GEMIN3 gene, the genotype TT carrier was associated with a 2.172-fold increased risk for pSS when compared with that of CT+CC carrier (odds ratio: 2.172, 95% CI, 1.133–4.166, p=0.019). Simultaneously, the pSS-susceptible TT carriers were associated with increased interferon-γ (IFN-γ) (P < 0.001) and tumor necrosis factor-α (TNF-α) (P = 0.003) levels when compared with that of CT+CC genotype carriers in female patients with pSS. The subsequent analysis also showed a weak positive correlation between IFN-γ and TNF-α levels (r=0.271, P = 0.019).

Conclusion

The predictors of GEMIN3 SNPs might modify pSS development in females by mediating the expression of miRNAs and therefore regulate the levels of IFN-γ and TNF-α.
背景:微RNA(miRNA)处理机制可通过改变miRNA的表达谱来改变原发性斯约格伦综合征(pSS)的发病风险。炎性细胞因子和活性氧(ROS)也与 pSS 有关;然而,miRNAs 表达的改变在其发病机制中的作用仍不清楚。我们旨在评估 miRNA 处理机制基因(包括 XPO5 (rs11077)、RAN (rs14035)、Dicer (rs3742330)、TNRC6B (rs9623117)、GEMIN3 (rs197412) 和 GEMIN4 (rs2740348))中的单核苷酸多态性 (SNPs) 与女性患者 pSS 风险之间的关系。此外,还评估了细胞因子和 ROS 与 pSS 易感 SNPs 的潜在关联:方法:在 74 名女性 pSS 患者和 77 名对照者中,对聚合酶链反应连接酶检测反应证实的 SNPs 进行基因分型。通过学生 t 检验、Wilcoxon 秩和检验、秩和检验、Pearson 相关性检验和二元逻辑回归分析对两者之间的关系进行了分析:GEMIN3 基因 rs197412 的基因型 TT 携带者与 CT+CC 携带者相比,患 pSS 的风险增加了 2.172 倍(几率比:2.172,95% CI,1.133-4.166,P=0.019)。同时,易感 pSS 的 TT 携带者与干扰素-γ(IFN-γ)的增加有关(PC结论:GEMIN3 SNPs的预测因子可能会通过介导miRNAs的表达来改变女性pSS的发展,从而调节IFN-γ和TNF-α的水平。
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引用次数: 0
Soluble RAGE attenuates myocardial I/R injury by suppressing interleukin-6 可溶性 RAGE 可通过抑制白细胞介素-6 减轻心肌 I/R 损伤。
IF 2.3 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-02-01 DOI: 10.1016/j.amjms.2024.08.001
Jie Zhang MD , Jian Liu MD , Jiming Yin MD , Xue Jiang MD , Lu Chen MD , Xiangjun Zeng PhD , Caixia Guo MD, PhD

Background

Inflammatory responses play a central role in myocardial ischemia/reperfusion (I/R) injury. Previous studies have demonstrated that the receptor for advanced glycation end-products (RAGE) is involved in the pro-inflammatory process of myocardial I/R injury by binding to diverse ligands. Thus, the inhibitory effects of soluble receptor for advanced glycation end-products (sRAGE), a decoy receptor for RAGE, on myocardial I/R injury may be associated with a reduced inflammatory state.

Methods

In this study, plasma levels of several inflammatory mediators were measured in patients with acute myocardial infarction (AMI) and I/R-treated cardiomyocyte-specific sRAGE knock-in (sRAGE-CKI) mice. Cardiac function, infarct size, and macrophage phenotypes were examined and documented in mouse hearts.

Results

We enrolled 38 patients diagnosed with myocardial infarction (AMI) [mean age, 58.81 ± 10.40 years] and 26 control with negative coronary arteriographic findings [mean age, 61.84 ± 8.57 years]. The results showed that sRAGE levels were significantly elevated in the AMI patient group compared with the control group (1905.00 [1462.50, 2332.5] vs 1570.00 [1335.00, 1800.00] pg/mL, p < 0.05), which were negatively correlated with interleukin (IL)-1, IL-6, and IL-8 levels. Cardiac-specific overexpression of sRAGE dramatically improved cardiac function and reduced infarct size during myocardial I/R. Furthermore, sRAGE overexpression decreased the plasma IL-6 levels and pro-inflammatory iNOS+ M1-macrophages, and increased CD206+ M2-macrophages in the mouse hearts.

Conclusions

Our findings suggested that sRAGE protects the heart from myocardial I/R injury by inhibiting the infiltration of pro-inflammatory M1-macrophages, and subsequently decreasing IL-6 secretion.
背景:炎症反应在心肌缺血/再灌注(I/R)损伤中起着核心作用。先前的研究表明,高级糖化终产物受体(RAGE)通过与多种配体结合参与了心肌缺血再灌注损伤的促炎过程。因此,RAGE 的诱饵受体可溶性高级糖化终产物受体(sRAGE)对心肌 I/R 损伤的抑制作用可能与炎症状态的减轻有关:本研究测定了急性心肌梗死(AMI)患者和经 I/R 处理的心肌细胞特异性 sRAGE 基因敲入(sRAGE-CKI)小鼠血浆中几种炎症介质的水平。对小鼠心脏的心功能、梗塞面积和巨噬细胞表型进行了检查和记录:我们招募了 38 名被诊断为心肌梗死(AMI)的患者(平均年龄为 58.81 ± 10.40 岁)和 26 名冠状动脉造影结果阴性的对照组患者(平均年龄为 61.84 ± 8.57 岁)。结果显示,与对照组相比,AMI 患者组的 sRAGE 水平显著升高(1905.00 [1462.50, 2332.5] vs 1570.00 [1335.00, 1800.00] pg/mL,p + M1-巨噬细胞,小鼠心脏中 CD206+ M2-巨噬细胞增多):我们的研究结果表明,sRAGE 可抑制促炎性 M1-巨噬细胞的浸润,进而减少 IL-6 的分泌,从而保护心脏免受心肌 I/R 损伤。
{"title":"Soluble RAGE attenuates myocardial I/R injury by suppressing interleukin-6","authors":"Jie Zhang MD ,&nbsp;Jian Liu MD ,&nbsp;Jiming Yin MD ,&nbsp;Xue Jiang MD ,&nbsp;Lu Chen MD ,&nbsp;Xiangjun Zeng PhD ,&nbsp;Caixia Guo MD, PhD","doi":"10.1016/j.amjms.2024.08.001","DOIUrl":"10.1016/j.amjms.2024.08.001","url":null,"abstract":"<div><h3>Background</h3><div>Inflammatory responses play a central role in myocardial ischemia/reperfusion (I/R) injury. Previous studies have demonstrated that the receptor for advanced glycation end-products (RAGE) is involved in the pro-inflammatory process of myocardial I/R injury by binding to diverse ligands. Thus, the inhibitory effects of soluble receptor for advanced glycation end-products (sRAGE), a decoy receptor for RAGE, on myocardial I/R injury may be associated with a reduced inflammatory state.</div></div><div><h3>Methods</h3><div>In this study, plasma levels of several inflammatory mediators were measured in patients with acute myocardial infarction (AMI) and I/R-treated cardiomyocyte-specific sRAGE knock-in (sRAGE-CKI) mice. Cardiac function, infarct size, and macrophage phenotypes were examined and documented in mouse hearts.</div></div><div><h3>Results</h3><div>We enrolled 38 patients diagnosed with myocardial infarction (AMI) [mean age, 58.81 ± 10.40 years] and 26 control with negative coronary arteriographic findings [mean age, 61.84 ± 8.57 years]. The results showed that sRAGE levels were significantly elevated in the AMI patient group compared with the control group (1905.00 [1462.50, 2332.5] vs 1570.00 [1335.00, 1800.00] pg/mL, <em>p</em> &lt; 0.05), which were negatively correlated with interleukin (IL)-1, IL-6, and IL-8 levels. Cardiac-specific overexpression of sRAGE dramatically improved cardiac function and reduced infarct size during myocardial I/R. Furthermore, sRAGE overexpression decreased the plasma IL-6 levels and pro-inflammatory iNOS<sup>+</sup> M1-macrophages, and increased CD206<sup>+</sup> M2-macrophages in the mouse hearts.</div></div><div><h3>Conclusions</h3><div>Our findings suggested that sRAGE protects the heart from myocardial I/R injury by inhibiting the infiltration of pro-inflammatory M1-macrophages, and subsequently decreasing IL-6 secretion.</div></div>","PeriodicalId":55526,"journal":{"name":"American Journal of the Medical Sciences","volume":"369 2","pages":"Pages 228-237"},"PeriodicalIF":2.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141904101","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
Acute urticaria and cardiovascular diseases 急性荨麻疹和心血管疾病。
IF 2.3 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-02-01 DOI: 10.1016/j.amjms.2024.08.004
Eli Magen MD , Eugene Merzon MD , Ilan Green MD , Israel Magen MD , Avivit Golan-Cohen MD , Shlomo Vinker MD , Ariel Israel MD, PhD

Background

Acute urticaria (AU), characterized by sudden skin wheals, has been associated with various triggers. While chronic urticaria's cardiovascular comorbidities have been studied, AU's associations remain largely unexplored.

Objective

This study aimed to identify prevalent cardiovascular comorbidities in AU patients and assess their clinical significance.

Methods

A retrospective cohort study used data from Leumit Health Services, matching AU patients with controls. Demographic, clinical, and laboratory data were analyzed. Statistical analyses included Fisher's Exact Test and Mann-Whitney U test.

Results

The AU group (72,851 individuals) showed no sex/ethnic distribution differences from controls (291,404 individuals). Systolic blood pressure, weight, BMI, glucose, hemoglobin A1c, C-reactive protein, eosinophil counts, total IgE levels were higher in AU (p < 0.001). Several cardiovascular comorbidities showed significant associations with AU, including valvular heart diseases (aortic regurgitation, mitral regurgitation, and pulmonary valve stenosis), cardiac arrhythmias (atrial fibrillation and others), deep vein thrombosis, diseases of capillaries, peripheral artery disease, cerebrovascular disease, coronary artery disease, and inflammatory heart diseases (pericarditis, heart failure, and hypertension) (p < 0.05). Medications, including antihistamines and glucocorticoids, were more prevalent in the AU group (p < 0.001).

Conclusion

This study's findings underscore the importance of recognizing cardiovascular comorbidities in AU patients and considering their implications for management. The observed associations provide insight into potential shared mechanisms between AU and cardiovascular diseases, though further research is needed to validate and expand upon these findings.
背景:急性荨麻疹(AU)以突发性皮肤喘息为特征,与各种诱发因素有关。虽然慢性荨麻疹的心血管并发症已得到研究,但急性荨麻疹的相关性在很大程度上仍未得到探讨:本研究旨在确定荨麻疹患者普遍存在的心血管并发症,并评估其临床意义:一项回顾性队列研究使用了 Leumit 健康服务机构的数据,将 AU 患者与对照组进行配对。研究分析了人口统计学、临床和实验室数据。统计分析包括费雪精确检验和曼-惠特尼U检验:AU组(72851人)与对照组(291404人)在性别/种族分布上没有差异。非盟患者的收缩压、体重、体重指数、血糖、血红蛋白 A1c、C 反应蛋白、嗜酸性粒细胞计数和总 IgE 水平均较高(P < 0.001)。一些心血管合并症与 AU 有显著相关性,包括瓣膜性心脏病(主动脉瓣反流、二尖瓣反流和肺动脉瓣狭窄)、心律失常(心房颤动等)、深静脉血栓、毛细血管疾病、外周动脉疾病、脑血管疾病、冠状动脉疾病和炎症性心脏病(心包炎、心力衰竭和高血压)(P < 0.05)。包括抗组胺药和糖皮质激素在内的药物在非盟组中更为普遍(P < 0.001):本研究的结果强调了认识到AU患者合并心血管疾病并考虑其对管理的影响的重要性。观察到的关联为了解非传染性疾病和心血管疾病之间的潜在共同机制提供了见解,尽管还需要进一步的研究来验证和扩展这些发现。
{"title":"Acute urticaria and cardiovascular diseases","authors":"Eli Magen MD ,&nbsp;Eugene Merzon MD ,&nbsp;Ilan Green MD ,&nbsp;Israel Magen MD ,&nbsp;Avivit Golan-Cohen MD ,&nbsp;Shlomo Vinker MD ,&nbsp;Ariel Israel MD, PhD","doi":"10.1016/j.amjms.2024.08.004","DOIUrl":"10.1016/j.amjms.2024.08.004","url":null,"abstract":"<div><h3>Background</h3><div>Acute urticaria (AU), characterized by sudden skin wheals, has been associated with various triggers. While chronic urticaria's cardiovascular comorbidities have been studied, AU's associations remain largely unexplored.</div></div><div><h3>Objective</h3><div>This study aimed to identify prevalent cardiovascular comorbidities in AU patients and assess their clinical significance.</div></div><div><h3>Methods</h3><div>A retrospective cohort study used data from Leumit Health Services, matching AU patients with controls. Demographic, clinical, and laboratory data were analyzed. Statistical analyses included Fisher's Exact Test and Mann-Whitney U test.</div></div><div><h3>Results</h3><div>The AU group (72,851 individuals) showed no sex/ethnic distribution differences from controls (291,404 individuals). Systolic blood pressure, weight, BMI, glucose, hemoglobin A1c, C-reactive protein, eosinophil counts, total IgE levels were higher in AU (p &lt; 0.001). Several cardiovascular comorbidities showed significant associations with AU, including valvular heart diseases (aortic regurgitation, mitral regurgitation, and pulmonary valve stenosis), cardiac arrhythmias (atrial fibrillation and others), deep vein thrombosis, diseases of capillaries, peripheral artery disease, cerebrovascular disease, coronary artery disease, and inflammatory heart diseases (pericarditis, heart failure, and hypertension) (p &lt; 0.05). Medications, including antihistamines and glucocorticoids, were more prevalent in the AU group (p &lt; 0.001).</div></div><div><h3>Conclusion</h3><div>This study's findings underscore the importance of recognizing cardiovascular comorbidities in AU patients and considering their implications for management. The observed associations provide insight into potential shared mechanisms between AU and cardiovascular diseases, though further research is needed to validate and expand upon these findings.</div></div>","PeriodicalId":55526,"journal":{"name":"American Journal of the Medical Sciences","volume":"369 2","pages":"Pages 160-165"},"PeriodicalIF":2.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141914929","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
Clinical characteristics of primary biliary cirrhosis - idiopathic inflammatory myopathy overlap syndrome: A single center study 原发性胆汁性肝硬化-特发性炎症性肌病重叠综合征的临床特征:一项单中心研究
IF 2.3 4区 医学 Q2 MEDICINE, GENERAL & INTERNAL Pub Date : 2025-02-01 DOI: 10.1016/j.amjms.2024.08.005
Juan Wu MD , Yue Yin MD, PhD , Xinxin Han MD, PhD , Hong Di MD, PhD , Yingdong Han MD, PhD , Min Shen MD, PhD , Yun Zhang MD , Xuejun Zeng MD, PhD

Purpose

Primary biliary cirrhosis–idiopathic inflammatory myopathy (PBC-IIM) overlap syndrome (OS) is a rare condition in which cardiac involvement is observed. We aimed to characterize the clinical features and associated factors of PBC-IIM OS patients with cardiac involvement.

Methods

Patients with PBC-IIM OS that visited our hospital from January 1983 to December 2021 were enrolled. Clinical presentations and laboratory and imaging data were recorded. The clinical data of patients with and without cardiac involvement were compared. According to the first instance of a disease flare, prognostic factors were also studied.

Results

Thirty-four patients with PBC-IIM OS were enrolled. A total of 58.8% of patients presented with muscle weakness at disease onset, which primarily involved skeletal muscle (85.3%). Slight liver dysfunction was discovered in this OS cohort. In patients with cardiac involvement, palpitation (63.6%) and dyspnea (36.4%) were the most common onset symptoms. Arrhythmia was a vital manifestation in OS patients, in which half of OS patients had nonsustained ventricular tachycardia (50.0%, 11/22). Compared with noncardiac involvement, myalgia (4.5%, P = 0.004) and fever (0.0%, P = 0.011) were reported relatively rarely at disease onset in the group with cardiac involvement. The prognosis analysis showed that positivity for anti-Ro52 (HR=0.00, P = 0.034) negatively correlated with relapse in OS patients.

Conclusion

PBC-IIM OS has unique features. Typical clinical manifestations and early worsening cardiac indicators can be used to identify cardiac involvement and predict prognosis. Anti-Ro52 may have prognostic value for PBC-IIM OS.
目的:原发性胆汁性肝硬化-特发性炎症性肌病(PBC-IIM)重叠综合征(OS)是一种罕见的心脏受累病症。我们的目的是描述心脏受累的 PBC-IIM OS 患者的临床特征和相关因素:方法:纳入1983年1月至2021年12月在我院就诊的PBC-IIM OS患者。记录临床表现、实验室和影像学数据。比较有心脏受累和无心脏受累患者的临床数据。根据疾病首次发作的情况,还研究了预后因素:34 名 PBC-IIM OS 患者入选。共有58.8%的患者在发病时出现肌无力,主要累及骨骼肌(85.3%)。在这组 OS 患者中发现了轻微的肝功能障碍。在心脏受累的患者中,心悸(63.6%)和呼吸困难(36.4%)是最常见的发病症状。心律失常是OS患者的重要表现,其中半数OS患者有非持续性室性心动过速(50.0%,11/22)。与非心脏受累相比,心脏受累组患者在发病时出现肌痛(4.5%,P=0.004)和发热(0.0%,P=0.011)的情况相对较少。预后分析显示,抗Ro52阳性(HR=0.00,P=0.034)与OS患者的复发呈负相关:结论:PBC-IIM OS具有独特的特征。结论:PBC-IIM OS具有独特的特征,典型的临床表现和早期恶化的心脏指标可用于识别心脏受累并预测预后。抗Ro52可能对PBC-IIM OS有预后价值。
{"title":"Clinical characteristics of primary biliary cirrhosis - idiopathic inflammatory myopathy overlap syndrome: A single center study","authors":"Juan Wu MD ,&nbsp;Yue Yin MD, PhD ,&nbsp;Xinxin Han MD, PhD ,&nbsp;Hong Di MD, PhD ,&nbsp;Yingdong Han MD, PhD ,&nbsp;Min Shen MD, PhD ,&nbsp;Yun Zhang MD ,&nbsp;Xuejun Zeng MD, PhD","doi":"10.1016/j.amjms.2024.08.005","DOIUrl":"10.1016/j.amjms.2024.08.005","url":null,"abstract":"<div><h3>Purpose</h3><div>Primary biliary cirrhosis–idiopathic inflammatory myopathy (PBC-IIM) overlap syndrome (OS) is a rare condition in which cardiac involvement is observed. We aimed to characterize the clinical features and associated factors of PBC-IIM OS patients with cardiac involvement.</div></div><div><h3>Methods</h3><div>Patients with PBC-IIM OS that visited our hospital from January 1983 to December 2021 were enrolled. Clinical presentations and laboratory and imaging data were recorded. The clinical data of patients with and without cardiac involvement were compared. According to the first instance of a disease flare, prognostic factors were also studied.</div></div><div><h3>Results</h3><div>Thirty-four patients with PBC-IIM OS were enrolled. A total of 58.8% of patients presented with muscle weakness at disease onset, which primarily involved skeletal muscle (85.3%). Slight liver dysfunction was discovered in this OS cohort. In patients with cardiac involvement, palpitation (63.6%) and dyspnea (36.4%) were the most common onset symptoms. Arrhythmia was a vital manifestation in OS patients, in which half of OS patients had nonsustained ventricular tachycardia (50.0%, 11/22). Compared with noncardiac involvement, myalgia (4.5%, <em>P</em> = 0.004) and fever (0.0%, <em>P</em> = 0.011) were reported relatively rarely at disease onset in the group with cardiac involvement. The prognosis analysis showed that positivity for anti-Ro52 (HR=0.00, <em>P</em> = 0.034) negatively correlated with relapse in OS patients.</div></div><div><h3>Conclusion</h3><div>PBC-IIM OS has unique features. Typical clinical manifestations and early worsening cardiac indicators can be used to identify cardiac involvement and predict prognosis. Anti-Ro52 may have prognostic value for PBC-IIM OS.</div></div>","PeriodicalId":55526,"journal":{"name":"American Journal of the Medical Sciences","volume":"369 2","pages":"Pages 166-175"},"PeriodicalIF":2.3,"publicationDate":"2025-02-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141914930","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":4,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
引用次数: 0
期刊
American Journal of the Medical Sciences
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