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Diagnostic accuracy of QuantiFERON-TB Gold Plus with Chemiluminescence Immunoassay: a systematic review and meta-analysis QuantiFERON-TB Gold Plus化学发光免疫测定的诊断准确性:系统综述和荟萃分析
IF 4.4 3区 医学 Q2 IMMUNOLOGY Pub Date : 2024-09-19 DOI: 10.1080/1744666x.2024.2407550
Shima Mahmoudi, Seyed Mohammad Sajad Hosseini Sharif
Tuberculosis (TB) remains a global health challenge, underscoring the need for accurate diagnosis, particularly for Latent TB Infection. This meta-analysis assesses the diagnostic performance of Qu...
结核病(TB)仍然是一项全球性的健康挑战,因此需要进行准确的诊断,尤其是对潜伏肺结核感染的诊断。这项荟萃分析评估了Qu...
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引用次数: 0
Complement as a major mediator of ANCA vasculitis and a target for precision therapy 补体是 ANCA 血管炎的主要介质和精准治疗的靶点
IF 4.4 3区 医学 Q2 IMMUNOLOGY Pub Date : 2024-09-18 DOI: 10.1080/1744666x.2024.2405170
Donna O. Bunch, Sarah E. Lewis, Hong Xiao, Peiqi Hu, J. Charles Jennette, Eveline Y. Wu
Complement was long thought not to be involved in ANCA vasculitis pathogenesis until studies in murine models demonstrated its central role. The current theory is ANCA-activated neutrophils degranu...
长期以来,人们一直认为补体与 ANCA 血管炎的发病机制无关,直到对小鼠模型的研究证明了补体的核心作用。目前的理论认为,ANCA激活的中性粒细胞在ANCA血管炎的发病机制中起着重要作用。
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引用次数: 0
Pioglitazone as a potential modulator in autoimmune diseases: a review on its effects in systemic lupus erythematosus, psoriasis, inflammatory bowel disease, and multiple sclerosis. 吡格列酮作为自身免疫性疾病的潜在调节剂:综述其对系统性红斑狼疮、银屑病、炎症性肠病和多发性硬化症的影响。
IF 3.9 3区 医学 Q2 IMMUNOLOGY Pub Date : 2024-09-16 DOI: 10.1080/1744666X.2024.2401614
Mohammad Esmail Nasrabadi, Ahmed Al-Harrasi, Saeed Mohammadi, Fateme Zarif Azam Kardani, Mina Rahmati, Ali Memarian

Introduction: Current medications for autoimmune disorders often induce broad-ranging side effects, prompting a growing interest in therapies with more specific immune system modulation. Pioglitazone, known for its anti-diabetic properties, is increasingly recognized for significant immunomodulatory potential. Beyond its traditional use in diabetes management, pioglitazone emerges as a promising therapeutic candidate for autoimmune disorders.

Areas covered: This comprehensive review explores pioglitazone's impact on four prominent autoimmune conditions: systemic lupus erythematosus, psoriasis, inflammatory bowel disease, and multiple sclerosis. We focus on pioglitazone's diverse effects on immune cells and cytokines in these diseases, highlighting its potential as a valuable therapeutic option for autoimmune diseases. Here we have reviewed the latest and most current research literature available on PubMed, based on research published in the last 15 years.

Expert opinion: Pioglitazone as an immunomodulatory agent can regulate T cell differentiation, inhibit inflammatory cytokines, and promote anti-inflammatory macrophages. While further clinical studies are needed to fully understand its mechanisms and optimize treatment strategies, pioglitazone represents a potential therapeutic approach to improve outcomes for patients with these challenging autoimmune conditions. The future of autoimmune disease research may involve personalized treatment approaches, and collaborative efforts to improve patient quality of life.

简介目前治疗自身免疫性疾病的药物通常会产生广泛的副作用,这促使人们对具有更强特异性免疫系统调节作用的疗法越来越感兴趣。吡格列酮以其抗糖尿病特性而闻名,其显著的免疫调节潜力也日益得到认可。除了在糖尿病治疗中的传统用途外,吡格列酮还是治疗自身免疫性疾病的一种有前途的候选疗法:本综述探讨了吡格列酮对四种主要自身免疫性疾病的影响:系统性红斑狼疮、银屑病、炎症性肠病和多发性硬化症。我们重点研究了吡格列酮对这些疾病中的免疫细胞和细胞因子的不同影响,强调了它作为自身免疫性疾病的重要治疗选择的潜力。在此,我们以过去 15 年发表的研究为基础,回顾了 PubMed 上最新的研究文献:吡格列酮作为一种免疫调节剂,可以调节T细胞分化、抑制炎症细胞因子、促进巨噬细胞抗炎。虽然还需要进一步的临床研究来全面了解其机制并优化治疗策略,但吡格列酮是一种潜在的治疗方法,可改善这些具有挑战性的自身免疫性疾病患者的预后。自身免疫性疾病研究的未来可能涉及个性化治疗方法和提高患者生活质量的合作努力。
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引用次数: 0
Innovative cell therapies for systemic sclerosis: available evidence and new perspectives 治疗系统性硬化症的创新细胞疗法:现有证据和新视角
IF 4.4 3区 医学 Q2 IMMUNOLOGY Pub Date : 2024-09-16 DOI: 10.1080/1744666x.2024.2402494
Alain Lescoat, Monalisa Ghosh, Stephan Kadauke, Dinesh Khanna
Systemic sclerosis (SSc) is the rheumatic disease with the highest individual mortality rate with detrimental impact on quality of life. Cell-based therapies may offer new perspectives for this dis...
系统性硬化症(SSc)是个人死亡率最高的风湿病,对患者的生活质量造成严重影响。以细胞为基础的疗法可为这种疾病的治疗提供新的视角...
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引用次数: 0
Single-cell RNA sequencing: an emerging tool revealing dysregulated innate and adaptive immune response at single cell level in Kawasaki disease. 单细胞 RNA 测序:揭示川崎病单细胞水平先天和适应性免疫反应失调的新兴工具。
IF 3.9 3区 医学 Q2 IMMUNOLOGY Pub Date : 2024-09-13 DOI: 10.1080/1744666X.2024.2401105
Saniya Sharma, Sumit Goel, Taru Goyal, Rakesh Kumar Pilania, Ridhima Aggarwal, Taranpreet Kaur, Manpreet Dhaliwal, Amit Rawat, Surjit Singh

Introduction: Kawasaki disease [KD] is a systemic disorder characterized by acute febrile illness due to widespread medium-vessel vasculitis, mainly affecting children. Despite the ongoing advanced research into the disease pathophysiology and molecular mechanisms, the exact etiopathogenesis of KD is still an enigma. Recently, single-cell RNA sequencing [scRNA-seq], has been utilized to elucidate the pathophysiology of KD at a resolution higher than that of previous methods.

Area covered: In the present article, we re-emphasize the pivotal role of this high-resolution technique, scRNA-seq, in the characterization of immune cell transcriptomic profile and signaling/response pathways in KD and explore the diagnostic, prognostic, and therapeutic potential of this new technique in KD. Using combinations of the search phrases 'KD, scRNA-seq, CAA, childhood vasculitis' a literature search was carried out on Scopus, Google Scholar, and PubMed until the beginning of 2024.

Expert opinion: scRNA-seq presents a transformative tool for dissecting KD at the cellular level. By revealing rare cell populations, gene expression alterations, and disease-specific pathways, scRNA-seq aids in understanding the intricacies of KD pathogenesis. This review will provide new insights into pathogenesis of KD and the field of applications of scRNA-seq in personalized therapeutics for KD in the future.

导言:川崎病(Kawasaki disease [KD])是一种以广泛中血管炎引起的急性发热为特征的全身性疾病,主要影响儿童。尽管对该病的病理生理学和分子机制的研究不断深入,但川崎病的确切发病机制仍是一个谜。最近,单细胞 RNA 测序(scRNA-seq)被用于阐明 KD 的病理生理学,其分辨率高于以往的方法:在本文中,我们再次强调了 scRNA-seq 这种高分辨率技术在鉴定 KD 中免疫细胞转录组特征和信号/反应通路中的关键作用,并探讨了这种新技术在 KD 中的诊断、预后和治疗潜力。使用 "KD、scRNA-seq、CAA、儿童脉管炎 "等搜索短语组合,在Scopus、Google Scholar和PubMed上进行了文献检索,直至2024年初。通过揭示罕见细胞群、基因表达改变和疾病特异性通路,scRNA-seq 有助于了解 KD 发病机制的复杂性。这篇综述将为 KD 的发病机制以及单细胞 RNA 测序在未来 KD 个性化疗法中的应用领域提供新的见解。
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引用次数: 0
Nociplastic pain in axial spondyloarthritis and psoriatic arthritis: role of JAK kinases in immunopathology and therapeutic impact of JAK inhibitors. 轴性脊柱关节炎和银屑病关节炎中的非关节痛:JAK 激酶在免疫病理学中的作用以及 JAK 抑制剂的治疗效果。
IF 3.9 3区 医学 Q2 IMMUNOLOGY Pub Date : 2024-09-08 DOI: 10.1080/1744666X.2024.2400294
Natalya Horbal, Walter P Maksymowych

Introduction: Pain in both peripheral and axial joints is a major symptom in patients with psoriatic arthritis (PsA) and axial spondyloarthritis (axSpA). Emerging evidence demonstrates pain mechanisms, beyond those related to inflammation or joint damage, based on aberrant processing of nociceptive stimuli peripherally as well as centrally. The Janus kinase/signal transducers and activators of transcription (JAK-STAT) signaling pathway has been implicated in the processing of pain beyond its role in mediating inflammation and inhibitors of this pathway approved for the treatment of axSpA and PsA have been shown to alleviate a broad array of pain outcomes in both axial and peripheral joints.

Areas covered: We review recent definitions and standardization of the nomenclature for categorizing chronic pain according to causality, assessment tools to evaluate nociplastic pain, the pathophysiologic role of JAK-STAT signaling in nociplastic pain, evidence for the presence of nociplastic pain in axSpA and PsA, and the impact of JAK inhibitors (JAKi) on pain outcomes in clinical trials (PubMed: 01/01/2019-04/01-2024).

Expert opinion: Nociplastic pain assessment has been confined almost entirely to the use of a limited number of questionnaires in cross-sectional studies of these diseases. Though effective for alleviating pain, it is unclear if JAKi specifically impact nociplastic pain.

导言:外周和轴关节疼痛是银屑病关节炎(PsA)和轴性脊柱关节炎(axSpA)患者的主要症状。新的证据表明,疼痛机制与炎症或关节损伤无关,而是基于外周和中枢对痛觉刺激的异常处理。Janus 激酶/信号转导和转录激活因子(JAK-STAT)信号通路在介导炎症的作用之外,还与疼痛的处理有牵连,已被批准用于治疗 axSpA 和 PsA 的该通路抑制剂已被证明可减轻轴关节和外周关节的一系列疼痛结果:我们回顾了根据因果关系对慢性疼痛进行分类的最新定义和术语标准化、评估非痉挛性疼痛的评估工具、JAK-STAT信号传导在非痉挛性疼痛中的病理生理作用、axSpA和PsA中存在非痉挛性疼痛的证据,以及临床试验中JAK抑制剂(JAKi)对疼痛结果的影响(PubMed:01/01/2019-04/01-2024):在这些疾病的横断面研究中,非关节性疼痛评估几乎完全局限于使用数量有限的调查问卷。尽管JAKi能有效缓解疼痛,但目前尚不清楚它是否会对非结节性疼痛产生具体影响。
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引用次数: 0
Predictive value analysis of albumin-related inflammatory markers for short-term outcomes in patients with In-hospital cardiac arrest. 白蛋白相关炎症指标对院内心脏骤停患者短期预后的预测价值分析。
IF 3.9 3区 医学 Q2 IMMUNOLOGY Pub Date : 2024-09-04 DOI: 10.1080/1744666X.2024.2399700
Linlin Xiao, Feng Li, Yuanhui Sheng, Xueping Hou, Xixi Liao, Pengfei Zhou, Yuping Qin, Xiaoying Chen, Jinglun Liu, Yetao Luo, Dong Peng, Shan Xu, Dan Zhang

Objective: This study investigated the predictive value of albumin-related inflammatory markers for short-term outcomes in in-hospital cardiac arrest (IHCA) patients.

Methods: A linear mixed model investigated the dynamic changes of markers within 72 hours after return of spontaneous circulation (ROSC). Time-Dependent COX regression explored the predictive value. Mediation analysis quantified the association of markers with organ dysfunctions and adverse outcomes.

Results: Prognostic Nutritional Index (PNI) and RDW-Albumin Ratio (RAR) slightly changed (p > 0.05). Procalcitonin-Albumin Ratio (PAR1) initially increased and then slowly decreased. Neutrophil-Albumin Ratio (NAR) and Platelet-Albumin Ratio (PAR2) decreased slightly during 24-48 hours (all p<0.05). PNI (HR = 1.646, 95%CI (1.033,2.623)), PAR1 (HR = 1.69, 95%CI (1.057,2.701)), RAR (HR = 1.752,95%CI (1.103,2.783)) and NAR (HR = 1.724,95%CI (1.078,2.759)) were independently associated with in-hospital mortality. PNI (PM = 45.64%, 95%CI (17.05%,87.02%)), RAR (PM = 45.07%,95%CI (14.59%,93.70%)) and NAR (PM = 46.23%,95%CI (14.59%,93.70%)) indirectly influenced in-hospital mortality by increasing SOFA (central) scores. PNI (PM = 21.75%, 95%CI(0.67%,67.75%)) may also indirectly influenced outcome by increasing SOFA (renal) scores (all p < 0.05).

Conclusions: Within 72 hours after ROSC, albumin-related inflammatory markers (PNI, PAR1, RAR, and NAR) were identified as potential predictors of short-term prognosis in IHCA patients. They may mediate the adverse outcomes of patients by causing damages to the central nervous system and renal function.

目的本研究探讨了白蛋白相关炎症指标对院内心脏骤停(IHCA)患者短期预后的预测价值:方法:线性混合模型研究了自发性循环恢复(ROSC)后 72 小时内指标的动态变化。时间依赖性 COX 回归探讨了预测价值。中介分析量化了指标与器官功能障碍和不良预后的关联:结果:预后营养指数(PNI)和RDW-白蛋白比值(RAR)略有变化(P > 0.05)。降钙素原-白蛋白比值(PAR1)最初上升,然后缓慢下降。中性粒细胞-白蛋白比率(NAR)和血小板-白蛋白比率(PAR2)在 24-48 小时内略有下降(均 p<0.05)。PNI(HR = 1.646,95%CI (1.033,2.623))、PAR1(HR = 1.69,95%CI (1.057,2.701))、RAR(HR = 1.752,95%CI (1.103,2.783))和 NAR(HR = 1.724,95%CI (1.078,2.759))与院内死亡率独立相关。PNI(PM = 45.64%,95%CI (17.05%,87.02%))、RAR(PM = 45.07%,95%CI (14.59%,93.70%))和 NAR(PM = 46.23%,95%CI (14.59%,93.70%))通过增加 SOFA(中心)评分间接影响院内死亡率。PNI(PM = 21.75%,95%CI(0.67%,67.75%))也可能通过增加SOFA(肾脏)评分间接影响预后(所有P<0.05):结论:在复苏后 72 小时内,白蛋白相关炎症标志物(PNI、PAR1、RAR 和 NAR)被确定为 IHCA 患者短期预后的潜在预测因子。它们可能会对中枢神经系统和肾功能造成损害,从而影响患者的不良预后。
{"title":"Predictive value analysis of albumin-related inflammatory markers for short-term outcomes in patients with In-hospital cardiac arrest.","authors":"Linlin Xiao, Feng Li, Yuanhui Sheng, Xueping Hou, Xixi Liao, Pengfei Zhou, Yuping Qin, Xiaoying Chen, Jinglun Liu, Yetao Luo, Dong Peng, Shan Xu, Dan Zhang","doi":"10.1080/1744666X.2024.2399700","DOIUrl":"10.1080/1744666X.2024.2399700","url":null,"abstract":"<p><strong>Objective: </strong>This study investigated the predictive value of albumin-related inflammatory markers for short-term outcomes in in-hospital cardiac arrest (IHCA) patients.</p><p><strong>Methods: </strong>A linear mixed model investigated the dynamic changes of markers within 72 hours after return of spontaneous circulation (ROSC). Time-Dependent COX regression explored the predictive value. Mediation analysis quantified the association of markers with organ dysfunctions and adverse outcomes.</p><p><strong>Results: </strong>Prognostic Nutritional Index (PNI) and RDW-Albumin Ratio (RAR) slightly changed (<i>p</i> > 0.05). Procalcitonin-Albumin Ratio (PAR1) initially increased and then slowly decreased. Neutrophil-Albumin Ratio (NAR) and Platelet-Albumin Ratio (PAR2) decreased slightly during 24-48 hours (all p<0.05). PNI (HR = 1.646, 95%CI (1.033,2.623)), PAR1 (HR = 1.69, 95%CI (1.057,2.701)), RAR (HR = 1.752,95%CI (1.103,2.783)) and NAR (HR = 1.724,95%CI (1.078,2.759)) were independently associated with in-hospital mortality. PNI (PM = 45.64%, 95%CI (17.05%,87.02%)), RAR (PM = 45.07%,95%CI (14.59%,93.70%)) and NAR (PM = 46.23%,95%CI (14.59%,93.70%)) indirectly influenced in-hospital mortality by increasing SOFA (central) scores. PNI (PM = 21.75%, 95%CI(0.67%,67.75%)) may also indirectly influenced outcome by increasing SOFA (renal) scores (all p < 0.05).</p><p><strong>Conclusions: </strong>Within 72 hours after ROSC, albumin-related inflammatory markers (PNI, PAR1, RAR, and NAR) were identified as potential predictors of short-term prognosis in IHCA patients. They may mediate the adverse outcomes of patients by causing damages to the central nervous system and renal function.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":" ","pages":"1-9"},"PeriodicalIF":3.9,"publicationDate":"2024-09-04","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142119347","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Resveratrol: immunological activity and possible application in children and adolescents with allergic rhinitis. 白藜芦醇:免疫活性及在儿童和青少年过敏性鼻炎患者中的可能应用。
IF 3.9 3区 医学 Q2 IMMUNOLOGY Pub Date : 2024-09-02 DOI: 10.1080/1744666X.2024.2399705
Matteo Naso, Chiara Trincianti, Lorenzo Drago, Maria Daglia, Giulia Brindisi, Francesco Paolo Brunese, Giulio Dinardo, Alessandra Gori, Cristiana Indolfi, Enrico Tondina, Attilio Varricchio, Anna Maria Zicari, Giorgio Ciprandi
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引用次数: 0
Updating on pregnancy in rheumatoid arthritis. 类风湿关节炎患者怀孕的最新情况。
IF 3.9 3区 医学 Q2 IMMUNOLOGY Pub Date : 2024-09-01 Epub Date: 2024-05-20 DOI: 10.1080/1744666X.2024.2356164
Carlotta Schenone, Greta Pacini, Emanuele Gotelli, Elvis Hysa, Rosanna Campitiello, Silvia Sammorì, Sabrina Paolino, Alberto Sulli, Maurizio Cutolo

Introduction: Rheumatoid arthritis (RA), the most prevalent autoimmune disease in reproductive years, exhibits a higher incidence in females, suggesting involvement of estrogens, genetics and environmental factors in disease onset. Literature shows smaller families in RA patients, driving increased interest in Assisted Reproductive Techniques.

Areas covered: This review elucidates how immunotolerance mechanisms contribute to favorable pregnancy outcomes in RA, emphasizing the need for a careful pregnancy planning to mitigate fetal complications and postnatal flares, which surpass those in the general population. A thorough medication evaluation, orchestrated by a multidisciplinary team, is imperative during pregnancy, weighing potential teratogenic effects against safer alternatives to balance medication safety with disease control. A systematic literature search on PubMed and MEDLINE, using specific terms, covered relevant academic journals up to the latest date.

Expert opinion: This narrative review comprehensively addresses pregnancy-related considerations in RA patients, prioritizing meticulous disease management with pregnancy and breastfeeding-compatible drugs in line with the latest recommendations and registry data. The focus remains on evaluating glucocorticoids, conventional, and biological disease-modifying drugs for compatibility during pregnancy and breastfeeding. Additionally, the evolving landscape of targeted synthetic drugs during pregnancy is explored, providing insights into the latest developments in rheumatological care.

导言:类风湿性关节炎(RA)是育龄期最常见的自身免疫性疾病,女性发病率较高,这表明发病与雌激素、遗传和环境因素有关。文献显示,RA 患者的家庭规模较小,这促使人们对辅助生殖技术越来越感兴趣:这篇综述阐明了免疫耐受机制如何有助于RA患者获得良好的妊娠结局,强调了谨慎制定妊娠计划的必要性,以减少胎儿并发症和产后复发,这些并发症和复发率超过了普通人群。在妊娠期间,由多学科团队协调进行全面的用药评估势在必行,权衡潜在的致畸效应和更安全的替代药物,在用药安全和疾病控制之间取得平衡。我们使用特定术语在 PubMed 和 MEDLINE 上进行了系统的文献检索,涵盖了截至最新日期的相关学术期刊:这篇叙述性综述全面阐述了RA患者妊娠相关的注意事项,根据最新建议和登记数据,优先考虑使用与妊娠和哺乳兼容的药物进行细致的疾病管理。重点仍然是评估糖皮质激素、常规和生物疾病修饰药物在妊娠和哺乳期间的兼容性。此外,该书还探讨了孕期靶向合成药物不断发展的情况,为风湿病护理的最新发展提供了见解。
{"title":"Updating on pregnancy in rheumatoid arthritis.","authors":"Carlotta Schenone, Greta Pacini, Emanuele Gotelli, Elvis Hysa, Rosanna Campitiello, Silvia Sammorì, Sabrina Paolino, Alberto Sulli, Maurizio Cutolo","doi":"10.1080/1744666X.2024.2356164","DOIUrl":"10.1080/1744666X.2024.2356164","url":null,"abstract":"<p><strong>Introduction: </strong>Rheumatoid arthritis (RA), the most prevalent autoimmune disease in reproductive years, exhibits a higher incidence in females, suggesting involvement of estrogens, genetics and environmental factors in disease onset. Literature shows smaller families in RA patients, driving increased interest in Assisted Reproductive Techniques.</p><p><strong>Areas covered: </strong>This review elucidates how immunotolerance mechanisms contribute to favorable pregnancy outcomes in RA, emphasizing the need for a careful pregnancy planning to mitigate fetal complications and postnatal flares, which surpass those in the general population. A thorough medication evaluation, orchestrated by a multidisciplinary team, is imperative during pregnancy, weighing potential teratogenic effects against safer alternatives to balance medication safety with disease control. A systematic literature search on PubMed and MEDLINE, using specific terms, covered relevant academic journals up to the latest date.</p><p><strong>Expert opinion: </strong>This narrative review comprehensively addresses pregnancy-related considerations in RA patients, prioritizing meticulous disease management with pregnancy and breastfeeding-compatible drugs in line with the latest recommendations and registry data. The focus remains on evaluating glucocorticoids, conventional, and biological disease-modifying drugs for compatibility during pregnancy and breastfeeding. Additionally, the evolving landscape of targeted synthetic drugs during pregnancy is explored, providing insights into the latest developments in rheumatological care.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":" ","pages":"1041-1052"},"PeriodicalIF":3.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140944451","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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
Can we define difficult-to-treat systemic sclerosis? 我们能否定义难以治疗的系统性硬化症?
IF 3.9 3区 医学 Q2 IMMUNOLOGY Pub Date : 2024-09-01 Epub Date: 2024-05-14 DOI: 10.1080/1744666X.2024.2352450
Gabriella Szűcs, Zoltán Szekanecz, Szilvia Szamosi

Introduction: Systemic sclerosis (SSc) is a chronic autoimmune rheumatic disease characterized by microvascular alterations, immunopathology, and widespread fibrosis involving various organs. It is considered difficult to treat due to several reasons: complex pathogenesis, heterogeneity, late diagnosis, limited treatment options for certain organ manifestations, lack of personalized medicine.

Areas covered: This review presents the heterogeneity, survival and organ manifestations with their risk factors of systemic sclerosis and their current treatment options, while drawing attention to difficult-to-treat forms of the disease, based on literature indexed in PubMed.

Expert opinion: Despite recent advances in the management of SSc over the last decades, the disease presents significant morbidity and mortality. Although available treatment protocols brought significant advancements in terms of survival in SSc-associated interstitial lung disease and pulmonary arterial hypertension, less success has been achieved in the treatment of Raynaud's phenomenon and digital ulcers and the results are modest in case of heart, gastrointestinal, and renal manifestations. There are patients who do not respond to treatment and deteriorate even with adequate therapy. They can be considered difficult-to treat (D2T) cases. We have created a possible score system based on the individual organ manifestations and highlighted treatment options for the D2T SSc category.

导言:系统性硬化症(SSc)是一种慢性自身免疫性风湿病,以微血管改变、免疫病理和广泛纤维化为特征,累及多个器官。由于发病机制复杂、异质性强、诊断较晚、某些器官表现的治疗方案有限、缺乏个性化医疗等原因,该病被认为难以治疗:本综述以PubMed索引的文献为基础,介绍了系统性硬化症的异质性、存活率、器官表现及其风险因素,以及目前的治疗方案,同时提请注意该病的难治形式:尽管过去几十年来系统性硬化症的治疗取得了最新进展,但该病的发病率和死亡率仍然很高。虽然现有的治疗方案大大提高了SSc相关间质性肺病和肺动脉高压患者的存活率,但在治疗雷诺现象和数字溃疡方面却收效甚微,而在治疗心脏、胃肠道和肾脏表现方面也收效甚微。有些患者对治疗没有反应,即使接受了适当的治疗,病情也会恶化。这些患者可被视为难治病例(D2T)。我们根据各个器官的表现建立了一个可能的评分系统,并强调了D2T SSc类别的治疗方案。
{"title":"Can we define difficult-to-treat systemic sclerosis?","authors":"Gabriella Szűcs, Zoltán Szekanecz, Szilvia Szamosi","doi":"10.1080/1744666X.2024.2352450","DOIUrl":"10.1080/1744666X.2024.2352450","url":null,"abstract":"<p><strong>Introduction: </strong>Systemic sclerosis (SSc) is a chronic autoimmune rheumatic disease characterized by microvascular alterations, immunopathology, and widespread fibrosis involving various organs. It is considered difficult to treat due to several reasons: complex pathogenesis, heterogeneity, late diagnosis, limited treatment options for certain organ manifestations, lack of personalized medicine.</p><p><strong>Areas covered: </strong>This review presents the heterogeneity, survival and organ manifestations with their risk factors of systemic sclerosis and their current treatment options, while drawing attention to difficult-to-treat forms of the disease, based on literature indexed in PubMed.</p><p><strong>Expert opinion: </strong>Despite recent advances in the management of SSc over the last decades, the disease presents significant morbidity and mortality. Although available treatment protocols brought significant advancements in terms of survival in SSc-associated interstitial lung disease and pulmonary arterial hypertension, less success has been achieved in the treatment of Raynaud's phenomenon and digital ulcers and the results are modest in case of heart, gastrointestinal, and renal manifestations. There are patients who do not respond to treatment and deteriorate even with adequate therapy. They can be considered difficult-to treat (D2T) cases. We have created a possible score system based on the individual organ manifestations and highlighted treatment options for the D2T SSc category.</p>","PeriodicalId":12175,"journal":{"name":"Expert Review of Clinical Immunology","volume":" ","pages":"1065-1081"},"PeriodicalIF":3.9,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140854850","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"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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Expert Review of Clinical Immunology
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