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Therapeutic strategies for primary heart involvement in systemic sclerosis. 系统性硬化症原发性心脏受累的治疗策略。
Pub Date : 2024-07-15 eCollection Date: 2024-06-01 DOI: 10.1515/rir-2024-0010
Veronica Batani, Lorenzo Dagna, Giacomo De Luca

Primary heart involvement (pHI) is frequent in systemic sclerosis (SSc), even though often underdiagnosed. SSc-pHI has been recently defined as cardiac abnormalities that are predominantly attributable to SSc rather than other causes and/or complications. SSc-pHI represents a major determinant of mortality in SSc, accounting alone for about 12% of disease-related deaths; its early recognition and promptly therapeutic interventions are therefore crucial. Both perfusion defects and myocardial inflammation contribute to the occurrence of myocardial fibrosis that precipitates myocardial remodeling, potentially leading to heart failure and arrhythmic complications. To date, clear evidence and guidelines for effectively managing SSc pHI are not established yet, resulting in a lack of a defined therapeutic algorithm. In this review we summarize the most recent scientific literature on the prevailing therapeutic strategies and interventions to manage SSc-pHI, with particular focus on therapeutic strategies to counteract the 3 major pathogenic events of the disease, i.e. microvascular damage, myocardial inflammation and myocardial fibrosis.

原发性心脏受累(pHI)在系统性硬化症(SSc)中很常见,但往往诊断不足。SSc-pHI 最近被定义为主要由 SSc 而非其他原因和/或并发症引起的心脏异常。SSc-pHI是决定SSc患者死亡率的一个主要因素,仅此一项就占疾病相关死亡的12%左右;因此,早期识别SSc-pHI并及时进行治疗干预至关重要。灌注缺损和心肌炎症都会导致心肌纤维化,进而引发心肌重塑,可能导致心力衰竭和心律失常并发症。迄今为止,有效治疗 SSc pHI 的明确证据和指南尚未确立,因此缺乏明确的治疗算法。在这篇综述中,我们总结了有关控制 SSc-pHI 的现行治疗策略和干预措施的最新科学文献,尤其侧重于对抗该疾病三大致病因素(即微血管损伤、心肌炎症和心肌纤维化)的治疗策略。
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引用次数: 0
Heart in a stone house: Systemic sclerosis with pericardial calcinosis. 石屋中的心脏伴有心包钙化的系统性硬化症。
Pub Date : 2024-07-15 eCollection Date: 2024-06-01 DOI: 10.1515/rir-2024-0017
Zhi Li, Mengying Zhang, Lanlan Jia, Chuanmiao Zhu, Junyuan Wang
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引用次数: 0
Outcomes of myocarditis in systemic sclerosis: A 3-year follow-up. 系统性硬化症心肌炎的预后:三年随访
Pub Date : 2024-07-15 eCollection Date: 2024-06-01 DOI: 10.1515/rir-2024-0015
Ajanee Mahakkanukrauh, Chingching Foocharoen, Narumol Chaosuwannakit, Siraphop Suwannaroj, Patnarin Pongkulkiat, Tippawan Onchan, Burabha Pussadhamma

Background and objectives: The clinical course, the outcomes of myocarditis, and the imaging progression of cardiac magnetic resonance imaging (MRI) in systemic sclerosis (SSc) are still unknown. We aimed at defining changes in cardiac MRI findings, the clinical course, and the outcomes of SSc patients previously defined as having myocarditis by cardiac MRI. Methods: This prospective cohort study included SSc patients, who had previously been diagnosed with myocarditis through cardiac MRI at the Scleroderma Clinic of Khon Kaen University, between 2018 and 2020 and had had annual follow-ups of cardiac MRI for at least 3 years. Data on demographics, clinical characteristics, cardiac MRI findings, treatment regimens, and outcomes were collected. Serial cardiac MRI on a yearly basis was analyzed to assess changes in myocardial involvement over the 3-year period.

Results: Ten SSc patients diagnosed with myocarditis via cardiac MRI were included. Most belonged to the diffuse cutaneous subset with a mean age of 58.3±8.6 years and were mildly symptomatic. Initial cardiac MRI findings showed myocardial edema and hyperemia in all patients and eight patients had had pre-existing myocardial scars, suggesting disease chronicity. Treatment for concomitant interstitial lung disease involved steroids with either cyclophosphamide or mycophenolate mofetil in 6 patients. Outcomes of myocarditis were stable, improving, and worsening in 4, 4, and 2 patients, respectively. There was no complete resolution of the cardiac MRI indices for myocarditis, and none had had major cardiac events.

Conclusion: Although SSc myocarditis on cardiac MRI may improve or show stability, the changes remained persistent. Among patients with SSc and mildly symptomatic myocarditis, the efficacy of steroids and immunosuppressive therapy is inconclusive. Over a 3-year follow-up, the prognosis had been acceptably good with no cardiac events.

背景和目的:系统性硬化症(SSc)患者的临床过程、心肌炎的预后以及心脏磁共振成像(MRI)的成像进展仍是未知数。我们的目的是确定心脏磁共振成像结果的变化、临床过程以及之前通过心脏磁共振成像被定义为患有心肌炎的 SSc 患者的预后。方法:这项前瞻性队列研究纳入了 2018 年至 2020 年期间曾在孔敬大学硬皮病诊所通过心脏磁共振成像诊断为心肌炎的 SSc 患者,他们每年都会接受心脏磁共振成像随访至少 3 年。我们收集了有关人口统计学、临床特征、心脏磁共振成像结果、治疗方案和结果的数据。每年对心脏磁共振成像进行序列分析,以评估3年期间心肌受累情况的变化:结果:纳入了10名通过心脏磁共振成像诊断为心肌炎的SSc患者。大多数患者属于弥漫性皮肤亚组,平均年龄(58.3±8.6)岁,症状轻微。最初的心脏磁共振成像结果显示,所有患者均存在心肌水肿和充血现象,其中八名患者的心肌原有疤痕,这表明该病具有长期性。6名患者因合并间质性肺部疾病而接受类固醇治疗,同时服用环磷酰胺或霉酚酸酯。4、4和2名患者的心肌炎结果分别为稳定、好转和恶化。心肌炎的心脏磁共振成像指标没有完全恢复,没有人发生重大心脏事件:结论:尽管心脏磁共振成像上的SSc心肌炎可能会有所改善或趋于稳定,但其变化仍然持续存在。结论:虽然SSc心肌炎在心脏磁共振成像上的表现可能会有所改善或趋于稳定,但变化仍然持续存在。在SSc和轻度症状性心肌炎患者中,类固醇和免疫抑制疗法的疗效尚无定论。在三年的随访中,患者的预后良好,没有发生任何心脏事件。
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引用次数: 0
Adalimumab in the management of psoriasis and psoriatic arthritis: Results from a Delphi investigation. 阿达木单抗在银屑病和银屑病关节炎治疗中的应用:德尔菲调查的结果。
Pub Date : 2024-03-31 eCollection Date: 2024-03-01 DOI: 10.1515/rir-2024-0006
Marco Matucci-Cerinic, Francesco Ciccia, Rosario Foti, Alessandro Giunta, Francesco Loconsole, Francesca Prignano, Rossana Scrivo, Giampiero Girolomoni

Background and objectives: Psoriasis (PsO) and psoriatic arthritis (PsA) are often undertreated and require a multidisciplinary approach. In recent years, patent expiration has allowed the introduction of tumor necrosis factor inhibitor (anti-TNF) biosimilars, which have stimulated a significant increase in the use of biological therapies. This article reports the findings of a multidisciplinary approach to achieve a consensus on the use of adalimumab in patients with PsO or PsA.

Methods: A voting panel of 36 Italian dermatologists and rheumatologists were chosen by eight Italian clinicians (the Board), to provide a consensus on the real-world management of PsO and PsA with adalimumab using the Delphi Method, comprising three survey rounds. Twelve statements were defined by the Board and submitted to the panel (rating scale 1-7).

Results: Clinicians reached a wide consensus on the effectiveness (score 6-7: 67%) and long-term efficacy (6-7: 100%) of adalimumab in all clinical forms of PsO and PsA, including pediatric patients (6-7: 85%). Considering cost-effectiveness and safety, adalimumab is suggested as a first-line treatment in patients with enthesitis, predominant peripheral arthritis, axial involvement or associated inflammatory bowel disease (IBD) or uveitis. Adalimumab can be also considered after failure of etanercept (6-7: 94%).

Conclusion: Results from this Delphi study clearly show an overall consensus on the use of adalimumab in the management of PsO and PsA, particularly as first-choice for specific subpopulations (uveitis, IBD, hidradenitis suppurativa). Considering the cost-effectiveness of biosimilars within Italy, adalimumab may represent an effective and safe first-line treatment for patients with moderate-to-severe PsO or PsA, and a valid choice for switching after failure.

背景和目的:银屑病(PsO)和银屑病关节炎(PsA)通常治疗不足,需要采用多学科方法治疗。近年来,由于专利到期,肿瘤坏死因子抑制剂(抗肿瘤坏死因子)生物仿制药得以问世,这刺激了生物疗法使用量的大幅增加。本文报告了一种多学科方法的研究结果,该方法旨在就阿达木单抗在PsO或PsA患者中的应用达成共识:由8名意大利临床医生(委员会)选出的36名意大利皮肤科医生和风湿病学家组成的投票小组,采用德尔菲法就使用阿达木单抗治疗PsO和PsA的实际情况达成共识,包括三轮调查。委员会确定了12项陈述,并提交给专家组(评分标准为1-7分):结果:临床医生就阿达木单抗对所有临床形式的PsO和PsA(包括儿童患者)的有效性(6-7分:67%)和长期有效性(6-7分:100%)达成了广泛共识(6-7分:85%)。考虑到成本效益和安全性,建议将阿达木单抗作为有关节炎、主要外周关节炎、轴性受累或伴有炎症性肠病(IBD)或葡萄膜炎的患者的一线治疗药物。在依那西普(6-7:94%)治疗失败后,也可考虑阿达木单抗:这项德尔菲研究的结果清楚地表明,在PsO和PsA的治疗中使用阿达木单抗,尤其是作为特定亚群(葡萄膜炎、IBD、化脓性扁桃体炎)的首选药物方面,已达成总体共识。考虑到意大利国内生物仿制药的成本效益,阿达木单抗可能是中重度PsO或PsA患者有效、安全的一线治疗药物,也是失败后转用的有效选择。
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引用次数: 0
Not all geriatric cachexia is cancer - The difficult lateonset rheumatoid arthritis. 并非所有老年恶病质都是癌症--难治的晚发性类风湿性关节炎。
Pub Date : 2024-03-31 eCollection Date: 2024-03-01 DOI: 10.1515/rir-2024-0009
Ana Rubim Correia, Inês Clara, Sara Raquel Martins, Tomás Fonseca
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引用次数: 0
Amyopathic dermatomyositis may be on the spectrum of autoinflammatory disease: A clinical review. 肌病皮肌炎可能属于自身炎症性疾病:临床回顾。
Pub Date : 2024-03-31 eCollection Date: 2024-03-01 DOI: 10.1515/rir-2024-0005
Saika Sharmeen, Lisa Christopher-Stine, Joann N Salvemini, Peter Gorevic, Richard Clark, Qingping Yao

Systemic autoinflammatory diseases (SAIDs) are distinct from autoimmune diseases. The former primarily results from abnormal innate immune response and genetic testing is crucial for disease diagnosis. Similar cutaneous involvement is a main feature for both SAID and dermatomyositis (DM), so they can be confused with each other. A literature search of PubMed and MEDLINE was conducted for relevant articles. The similarities and differences between these two types of diseases were analyzed. We found phenotypic similarities between these two types of disorders. Accumulating data supports a major role of the innate immune system and a similar cytokine profile. Molecular testing using an autoinflammatory disease gene panel may help identify SAID patients from the DM population and may offer therapeutic benefit using interleukin-1 (IL-1) inhibitors. A subset of DM, notably amyopathic dermatomyositis in the absence of autoantibodies may be on the spectrum of autoinflammatory disease.

系统性自身炎症疾病(SAIDs)有别于自身免疫性疾病。前者主要是先天性免疫反应异常所致,基因检测是疾病诊断的关键。类似的皮肤受累是 SAID 和皮肌炎(DM)的主要特征,因此两者容易混淆。我们对 PubMed 和 MEDLINE 上的相关文章进行了文献检索。分析了这两种疾病的异同。我们发现这两类疾病在表型上有相似之处。不断积累的数据支持先天性免疫系统的主要作用和相似的细胞因子谱。使用自身炎症性疾病基因面板进行的分子检测可能有助于从 DM 群体中识别出 SAID 患者,使用白细胞介素-1(IL-1)抑制剂可能会给治疗带来益处。DM 的一个亚群,尤其是无自身抗体的肌病性皮肌炎,可能属于自身炎症性疾病的范畴。
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引用次数: 0
Early diagnosis and standardized treatment are critical to improve the prognosis of patients with Takayasu's arteritis. 早期诊断和规范治疗对于改善高安动脉炎患者的预后至关重要。
Pub Date : 2024-03-31 eCollection Date: 2024-03-01 DOI: 10.1515/rir-2024-0001
Xinping Tian, Xiaofeng Zeng
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引用次数: 0
The effect of anti-TNF drugs on the intestinal microbiota in patients with spondyloarthritis, rheumatoid arthritis, and inflammatory bowel diseases. 抗肿瘤坏死因子药物对脊柱关节炎、类风湿性关节炎和炎症性肠病患者肠道微生物群的影响。
Pub Date : 2024-03-31 eCollection Date: 2024-03-01 DOI: 10.1515/rir-2024-0003
Francesco Ciccia, Nikolas Konstantine Dussias, Saviana Gandolfo, Fernando Rizzello, Paolo Gionchetti

Spondyloarthritis (SpA), rheumatoid arthritis (RA), and inflammatory bowel diseases (IBD) are chronic inflammatory autoimmune diseases that are associated with alterations in the composition of the intestinal microbiota (i.e., dysbiosis). For SpA and RA, a gut-joint-enthesis axis is hypothesized and recent data suggests that dysbiosis may contribute directly to initiating and perpetuating joint and spine inflammation. Biologic drugs targeting tumor necrosis factor (TNF) are effective in treating these diseases and have been shown to partially restore the disrupted microbiome. Hence, drugs that affect both the intestinal and joint components of these diseases, such as anti-TNF drugs, may act on the intestinal microbiome. However, despite the remarkable efficacy of anti-TNF-α treatments, non-responders are frequent, and predictors of patient outcomes have not been identified. In this narrative review, we summarize recent research on the downstream effects of anti-TNF drugs on the intestinal microbiota in SpA, RA, and IBD. We also discuss whether these changes could have a role as predictive biomarkers of anti-TNF response.

脊柱关节炎(Spondyloarthritis,SPA)、类风湿性关节炎(Rheumatoid arthritis,RA)和炎症性肠病(Inflammatory bowel diseases,IBD)都是慢性炎症性自身免疫性疾病,与肠道微生物群组成的改变(即菌群失调)有关。就 SpA 和 RA 而言,肠道-关节-假体轴是一种假设,最近的数据表明,菌群失调可能直接导致关节和脊柱炎症的发生和延续。以肿瘤坏死因子(TNF)为靶点的生物药物可有效治疗这些疾病,并已证明可部分恢复紊乱的微生物群。因此,同时影响这些疾病的肠道和关节部分的药物,如抗 TNF 药物,可能会作用于肠道微生物群。然而,尽管抗肿瘤坏死因子-α疗法疗效显著,但无应答者屡见不鲜,而且患者预后的预测因素尚未确定。在这篇叙述性综述中,我们总结了抗肿瘤坏死因子药物对 SpA、RA 和 IBD 患者肠道微生物群下游效应的最新研究。我们还讨论了这些变化是否可作为抗肿瘤坏死因子反应的预测性生物标志物。
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引用次数: 0
The vanishing touch: Unveiling the tuft erosion in scleroderma. 消失的触感揭开硬皮病簇状侵蚀的神秘面纱。
Pub Date : 2024-03-31 eCollection Date: 2024-03-01 DOI: 10.1515/rir-2024-0008
Hui Jiang, Yangzhong Zhou
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引用次数: 0
Clinical implications of seropositive and seronegative autoantibody status in rheumatoid arthritis patients: A comparative multicentre observational study. 类风湿性关节炎患者血清阳性和血清阴性自身抗体状态的临床意义:一项多中心观察比较研究。
Pub Date : 2024-03-31 eCollection Date: 2024-03-01 DOI: 10.1515/rir-2024-0007
Nevin Hammam, Passant N El-Husseiny, Suzan S Al-Adle, Nermeen Samy, Nora Y Elsaid, Dina F El-Essawi, Eman F Mohamed, Samar M Fawzy, Samah A El Bakry, Maha Nassr, Samah I Nasef, Hanan M El-Saadany, Shereen Elwan, Nada M Gamal, Abdelhfeez Moshrif, Osman Hammam, Rawhya R El Shereef, Faten Ismail, Samar Tharwat, Doaa Mosad Mosa, Mervat I Abd Elazeem, Enas A Abdelaleem, Tamer A Gheita

Background and objectives: Rheumatoid factor (RF) and anti-cyclic citrullinated protein (anti-CCP) have been used to improve the diagnosis and prognosis of rheumatoid arthritis (RA). However, their association with RA disease phenotypes, individually and in combination, is not well studied. The aim of the study was to compare patients' and disease characteristics, activity and severity in double seronegative (DNRA), single seropositive RF, single seropositive anti-CCP and double seropositive (DPRA) patients.

Methods: Adults subjects with RA from Egyptian College of Rheumatology (ECR) database who had RF and anti-CCP results available were included. Demographic, clinical features, disease activity score 28 (DAS28), Health Assessment Questionnaire (HAQ) and laboratory data were collected and compared among different RA groups.

Results: 5268 RA patients with mean age of 44.9±11.6 years, and 4477 (85%) were females. 2900 (55%) had DPRA, 892 (16.9%) had single positive RF, 597 (11.3%) had single positive anti-CCP while 879 (16.7%) had DNRA. Patients with DPRA had significantly high percentage of metabolic syndrome (19.3%, P < 0.001), and functional impairment using HAQ (P = 0.01). Older age (RRR [relative risk ratio]: 1.03, 95%CI: 1.0, 1.0, P = 0.029), greater DAS28 (RRR: 1.51, 95%CI: 1.2, 1.9, P < 0.001), higher steroid use (RRR: 2.4, 95%CI: 1.36, 4.25, P = 0.002) were at higher risk of DPRA while longer disease duration (RRR: 1.08, 95%CI: 1.01, 1.16, P = 0.017) and fibromyalgia syndrome (RRR: 2.54, 95%CI: 1.10, 5.88, P = 0.028) were associated with higher odds of single positive RF status.

Conclusion: Dual antibody-positive status has higher disease activity and severity, and higher chance of development of metabolic syndrome; highlighting the implicated role of inflammation, atherogenesis and cardiovascular disease risk in RA.

背景和目的:类风湿因子(RF)和抗环瓜氨酸蛋白(anti-CCP)已被用于改善类风湿性关节炎(RA)的诊断和预后。然而,对它们单独或联合使用与 RA 疾病表型的关系研究不多。本研究旨在比较双血清阴性(DNRA)、单血清阳性 RF、单血清阳性抗CCP 和双血清阳性(DPRA)患者的病情特征、活动性和严重程度:方法:纳入埃及风湿病学会(ECR)数据库中具有RF和抗CCP结果的成人RA患者。收集人口统计学、临床特征、疾病活动度评分 28(DAS28)、健康评估问卷(HAQ)和实验室数据,并对不同 RA 组别进行比较:结果:5268 名 RA 患者,平均年龄为(44.9±11.6)岁,其中 4477 名(85%)为女性。2900例(55%)为DPRA,892例(16.9%)为RF单项阳性,597例(11.3%)为抗CCP单项阳性,879例(16.7%)为DNRA。DPRA 患者患有代谢综合征(19.3%,P < 0.001)和 HAQ 功能障碍(P = 0.01)的比例明显较高。年龄越大(RRR [相对风险比]:1.03,95%CI:1.0,1.0,P = 0.029)、DAS28 越高(RRR:1.51,95%CI:1.2,1.9,P < 0.001)、使用类固醇越多(RRR:2.4,95%CI:1.36,4.25,P = 0.002)患 DPRA 的风险较高,而病程较长(RRR:1.08,95%CI:1.01,1.16,P = 0.017)和纤维肌痛综合征(RRR:2.54,95%CI:1.10,5.88,P = 0.028)与 RF 单项阳性状态的几率较高相关:结论:双重抗体阳性者的疾病活动度和严重程度较高,患代谢综合征的几率也较高;这突出表明了炎症、动脉粥样硬化和心血管疾病风险在RA中的作用。
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引用次数: 0
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Rheumatology and immunology research
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