Best Practice & Research in Clinical Rheumatology最新文献

英文中文

Emerging therapies in idiopathic inflammatory myopathies: Advances in treatment and future directions 特发性炎症性肌病的新疗法：治疗进展和未来方向。

IF 4.8 2区医学 Q1 RHEUMATOLOGY

Best Practice & Research in Clinical Rheumatology

Pub Date : 2025-12-01 DOI: 10.1016/j.berh.2025.102107

Laure Gallay , Eaman Alhassan , Niladri Bhowmick , Rohit Aggarwal

Idiopathic inflammatory myopathies are a group of rare autoimmune diseases characterized by chronic muscle inflammation and variable systemic involvement. Although conventional therapies, including corticosteroids and immunosuppressants, form the cornerstone of treatment, most patients do not achieve remission, underscoring the need for more effective options. Advances in understanding disease pathogenesis have led to the development of novel targeted therapies. Strategies aimed at disrupting key immune pathways, such as interferon signaling, T cell activation, and B cell maturation, have shown promising results. Additionally, innovative approaches such as engineered cellular therapies are currently explored. These treatments offer the potential to achieve deeper and more durable disease control, reduce corticosteroid dependence, and improve quality of life for the patients. In this chapter, we present the recent findings on novel therapies for the treatment of IIM (all subtypes except IBM) by discussing the rationale behind their use, their potential role in improving patient outcomes in the different IIM subtypes, as well as the limitations of their wide implementation. Future strategies and promising therapies under investigation are also highlighted.

特发性炎症性肌病是一组罕见的自身免疫性疾病，其特征是慢性肌肉炎症和不同的全身累及。尽管包括皮质类固醇和免疫抑制剂在内的常规疗法是治疗的基础，但大多数患者并没有达到缓解，这强调了需要更有效的选择。对疾病发病机制的理解的进步导致了新的靶向治疗的发展。旨在破坏关键免疫通路的策略，如干扰素信号、T细胞激活和B细胞成熟，已经显示出有希望的结果。此外，创新的方法，如工程细胞疗法目前正在探索。这些治疗方法有可能实现更深入、更持久的疾病控制，减少对皮质类固醇的依赖，并改善患者的生活质量。在本章中，我们通过讨论其使用背后的基本原理，它们在改善不同IIM亚型患者预后方面的潜在作用，以及它们广泛实施的局限性，介绍了治疗IIM（除IBM外的所有亚型）的新疗法的最新发现。未来的策略和有前途的治疗正在研究中也强调。

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引用次数: 0

Novel therapies – IMID Co-morbidities 新疗法- IMID合并症。

IF 4.8 2区医学 Q1 RHEUMATOLOGY

Best Practice & Research in Clinical Rheumatology

Pub Date : 2025-12-01 DOI: 10.1016/j.berh.2025.102089

Tsz On Lam, Ho So, Lai-Shan Tam

Cardiovascular diseases (CVD) and metabolic syndrome are common in patients with immune-mediated inflammatory diseases (IMID). These conditions exhibit significant interactions, between diseases and in relation to treatments, highlighting the need for rheumatologists to take an active role in their management. It is now known that inflammation mediated by adipokines is important in the pathogenesis of obesity and cardiovascular-kidney-metabolic (CKM) syndrome. Novel therapeutics developed for metabolic comorbidities have demonstrated pleiotropic benefit across a range of diseases. Some of them even possess anti-inflammatory and immuno-modulatory effects, serving as adjuncts to the management of IMID. On the other hand, anti-inflammatory drugs have been rigorously tested for their efficacy in CVD and colchicine has become the first anti-inflammatory drug approved for this purpose. This paper will focus on the novel therapeutics and new purposes of conventional drugs in the management of CVD and metabolic syndrome, with additional discussion of their relevance in IMID.

心血管疾病（CVD）和代谢综合征在免疫介导性炎症性疾病（IMID）患者中很常见。这些情况表现出显著的相互作用，疾病之间和相关的治疗，突出需要风湿病学家采取积极的作用，在他们的管理。现在已经知道，由脂肪因子介导的炎症在肥胖和心肾代谢综合征的发病机制中起重要作用。针对代谢合并症开发的新疗法在一系列疾病中显示出多效性益处。其中一些甚至具有抗炎和免疫调节作用，作为治疗IMID的辅助手段。另一方面，抗炎药物对心血管疾病的疗效已经经过严格的测试，秋水仙碱已经成为第一个被批准用于这一目的的抗炎药物。本文将重点介绍传统药物在心血管疾病和代谢综合征治疗中的新疗法和新用途，并进一步讨论它们与IMID的相关性。

引用次数: 0

Pipeline treatments on psoriatic disease 银屑病的管道治疗。

IF 4.8 2区医学 Q1 RHEUMATOLOGY

Best Practice & Research in Clinical Rheumatology

Pub Date : 2025-12-01 DOI: 10.1016/j.berh.2025.102090

André L. Ribeiro , Leandro L. Leite , Raphael Micheroli , Daniela B. Tovar-Batisdas , Maria-Angeliki Gkini , Fabian Proft

Psoriatic disease is a heterogeneous and multifaceted disease affecting musculoskeletal, dermatologic, and systemic domains, with low sustained remission rates despite advances in therapy. Current treatments, primarily targeting TNF, IL-17, IL-23, PDE4 inhibitors and JAK-STAT pathways, remain insufficient for many patients, highlighting the need for novel therapeutic approaches. This review explores pipeline treatments in psoriatic disease, including new JAK-STAT inhibitors (TYK2, JAK1), next-generation IL-17 inhibitors, IL-23-targeted therapies, and novel immune-modulating agents. The emerging role of combination therapy is also discussed, with dual biologic and small-molecule approaches showing potential in refractory disease. Additionally, microbiome-targeted therapies and metabolic interventions, including probiotics and GLP-1 receptor agonists, are being investigated as adjunctive strategies to improve disease control. While these innovations offer exciting opportunities for personalized medicine, challenges remain regarding long-term safety, optimal treatment sequencing, and combination strategies. Further randomized controlled trials and real-world data are necessary to define the most effective and sustainable treatment approaches for psoriatic disease.

银屑病是一种异质性和多方面的疾病，影响肌肉骨骼、皮肤和全身领域，尽管治疗取得了进展，但持续缓解率很低。目前的治疗方法主要针对TNF、IL-17、IL-23、PDE4抑制剂和JAK-STAT通路，对许多患者来说仍然不足，这突出了对新治疗方法的需求。本文综述了银屑病的管道治疗，包括新的JAK-STAT抑制剂（TYK2, JAK1），下一代IL-17抑制剂，il -23靶向治疗和新的免疫调节剂。本文还讨论了联合治疗的新作用，双生物和小分子方法在难治性疾病中显示出潜力。此外，微生物组靶向治疗和代谢干预，包括益生菌和GLP-1受体激动剂，正在研究作为改善疾病控制的辅助策略。虽然这些创新为个性化医疗提供了令人兴奋的机会，但在长期安全性、最佳治疗顺序和联合策略方面仍然存在挑战。需要进一步的随机对照试验和真实世界的数据来确定银屑病最有效和可持续的治疗方法。

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引用次数: 0

Novel therapies in Sjögren’s disease: A systematic review of the literature Sjögren病的新疗法：文献系统综述

IF 4.8 2区医学 Q1 RHEUMATOLOGY

Best Practice & Research in Clinical Rheumatology

Pub Date : 2025-12-01 DOI: 10.1016/j.berh.2025.102084

Cristina Pelkas , Kyle B. Franke , Fabien B. Vincent , Maureen Rischmueller

Sjögren's disease (SjD) is a chronic systemic autoimmune disorder that primarily involves lymphocytic infiltration of exocrine glands, with frequent extra-glandular manifestations. Historically, treatment options for SjD have been limited to alleviating symptoms, rather than treating the underlying cause or preventing disease progression. Furthermore, past clinical trials of therapies such as rituximab failed to demonstrate improvement in symptoms or disease activity. Recently, novel therapeutic strategies targeting underlying disease pathogenesis – including transcription factors, circulating RNA, and B and T cell activity – herald a paradigm shift. Given the complexities of diagnosis, clinical assessment and treatment in SjD, improved clinical trial design with enhanced patient stratification, greater inclusivity and better outcome measures are paramount in evaluating new therapeutics. This systematic review aims to provide a comprehensive overview of recent SjD therapeutic advances, assess trial inclusivity with respect to sex/gender and ethnicity, critically examine negative pivotal trials and highlight promising directions for future research.

Sjögren's disease （SjD）是一种慢性系统性自身免疫性疾病，主要涉及外分泌腺的淋巴细胞浸润，具有常见的腺外表现。从历史上看，SjD的治疗选择仅限于缓解症状，而不是治疗根本原因或预防疾病进展。此外，过去的临床试验，如利妥昔单抗未能证明改善症状或疾病活动。最近，针对潜在疾病发病机制的新治疗策略——包括转录因子、循环RNA、B细胞和T细胞活性——预示着范式的转变。考虑到SjD的诊断、临床评估和治疗的复杂性，改进临床试验设计，增强患者分层，更大的包容性和更好的结果测量在评估新疗法中至关重要。本系统综述旨在提供近期SjD治疗进展的全面概述，评估性别/性别和种族方面的试验包容性，批判性地检查负面关键试验，并强调未来研究的有希望的方向。

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引用次数: 0

Novel therapies in SSc SSc的新疗法。

IF 4.8 2区医学 Q1 RHEUMATOLOGY

Best Practice & Research in Clinical Rheumatology

Pub Date : 2025-12-01 DOI: 10.1016/j.berh.2025.102104

Janet Pope

Systemic sclerosis (SSc) is an autoimmune connective tissue disease that is rare and targets fibrosis and vasculopathy with autoantibodies present. There are guidelines in SSc that are evidence based and can facilitate appropriate treatment for many patients with SSc. However, there is no cure and in many patients the quality and quantity of life are significantly affected. Thus, novel therapies in SSc are warranted in order to try to prevent damage and increase quality of life and improve survival. This update will expand where the standard of care treatment in SSc may be enhanced by novel therapies and recent or ongoing trials. As few studies of Raynaud's phenomenon, digital ulcers, gastrointestinal, cardiac, renal and musculoskeletal systems in SSc are ongoing, they will not be included. This paper will concentrate on immune modification for skin involvement and interstitial lung disease and pulmonary hypertension. Potentially transformative treatments will be highlighted and where they may fit into a future improved standard of care for SSc patients.

系统性硬化症（SSc）是一种罕见的自身免疫性结缔组织疾病，以自身抗体存在的纤维化和血管病变为靶点。SSc有一些基于证据的指南，可以促进许多SSc患者的适当治疗。然而，此病无法治愈，许多患者的生活质量和数量都受到严重影响。因此，为了防止损伤、提高生活质量和提高生存率，SSc的新疗法是必要的。这一更新将扩大SSc的护理标准治疗可能通过新疗法和最近或正在进行的试验得到加强的领域。由于目前正在进行的关于雷诺现象、数字溃疡、胃肠道、心脏、肾脏和肌肉骨骼系统的研究很少，因此将不包括在内。本文将集中讨论皮肤受累、间质性肺疾病和肺动脉高压的免疫修饰。将强调潜在的变革性治疗方法，以及它们可能适合未来改进的SSc患者护理标准的地方。

引用次数: 0

Imaging in sarcoid disease 结节病的影像学。

IF 4.8 2区医学 Q1 RHEUMATOLOGY

Best Practice & Research in Clinical Rheumatology

Pub Date : 2025-09-01 DOI: 10.1016/j.berh.2025.102054

Sherief Ghozy , Jonathan W. Revels , Aakanksha Sriwastwa , Lily L. Wang , Jennifer S. Weaver , Sherry S. Wang

Sarcoidosis is a complex multisystem inflammatory disease characterized by noncaseating granulomas and variable clinical manifestations, most commonly affecting the lungs, skin, heart, and nervous system. Imaging is central in its diagnosis, staging, and management, providing essential insights into organ involvement and disease activity. Pulmonary manifestations remain the hallmark, with modalities such as high-resolution chest computed tomography (CT) and chest radiography offering critical diagnostic clues. Imaging techniques, including Fluorodeoxyglucose Positron Emission Tomography (FDG-PET) and cardiac magnetic resonance imaging, are invaluable for identifying cardiac and systemic involvement, including cutaneous and musculoskeletal, while abdominal MRI and ultrasound help delineate hepatic and splenic manifestations. Neurosarcoidosis requires MRI for precise evaluation, supplemented by FDG-PET to guide biopsy and monitor treatment response. This chapter synthesizes the imaging features of sarcoidosis across organ systems, emphasizing practical approaches to diagnosis and management while identifying key areas for future research.

结节病是一种复杂的多系统炎症性疾病，以非干酪化肉芽肿为特征，临床表现多变，最常累及肺、皮肤、心脏和神经系统。影像是诊断、分期和治疗的核心，提供了器官受累和疾病活动的基本见解。肺部表现仍然是该病的标志，高分辨率胸部计算机断层扫描（CT）和胸部x线摄影提供了关键的诊断线索。成像技术，包括氟脱氧葡萄糖正电子发射断层扫描（FDG-PET）和心脏磁共振成像，对于识别心脏和全身病变（包括皮肤和肌肉骨骼）是无价的，而腹部MRI和超声有助于描绘肝脏和脾脏的表现。神经结节病需要MRI进行精确评估，辅以FDG-PET指导活检和监测治疗反应。本章综合了结节病跨器官系统的影像学特征，强调了实用的诊断和管理方法，同时确定了未来研究的关键领域。

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引用次数: 0

Imaging of crystal-induced arthropathies in 2025 2025年晶体诱发关节病的影像学研究。

IF 4.8 2区医学 Q1 RHEUMATOLOGY

Best Practice & Research in Clinical Rheumatology

Pub Date : 2025-09-01 DOI: 10.1016/j.berh.2025.102063

Silvia Sirotti , Tristan Pascart , Ralf Thiele , Georgios Filippou

In recent years, imaging has become an essential tool in the assessment of crystal-induced arthropathies (CIAs), including gout, calcium pyrophosphate deposition disease, and basic calcium phosphate crystal deposition. Advances in imaging have improved diagnosis and disease monitoring, leading to its integration into classification criteria and clinical guidelines.

Ultrasound (US), conventional radiography (CR), and dual-energy computed tomography (DECT) each offer unique advantages. US is a widely accessible, cost-effective, and dynamic tool, while DECT provides crystal-specific images, aiding particularly in gout diagnosis. CR, though less sensitive to early crystal deposition, remains valuable for evaluating structural damage and chronic changes.

Despite these advances, challenges remain. The specificity and sensitivity of imaging findings need further validation, and the clinical relevance of certain imaging features is debated. This review summarizes recent developments, highlights key strengths, and discusses unresolved issues, emphasizing areas where future research is needed to optimize imaging use in CIAs.

近年来，影像学已成为评估晶体性关节病（CIAs）的重要工具，包括痛风、焦磷酸钙沉积病和碱性磷酸钙晶体沉积。影像学的进步改善了诊断和疾病监测，使其纳入分类标准和临床指南。超声（US）、常规放射照相（CR）和双能计算机断层扫描（DECT）各有其独特的优势。US是一种广泛可及的、具有成本效益的动态工具，而DECT提供晶体特异性图像，特别有助于痛风诊断。CR虽然对早期晶体沉积不太敏感，但在评估结构损伤和慢性变化方面仍有价值。尽管取得了这些进步，但挑战依然存在。影像学结果的特异性和敏感性需要进一步验证，某些影像学特征的临床相关性存在争议。这篇综述总结了最近的发展，突出了关键优势，并讨论了尚未解决的问题，强调了未来需要研究的领域，以优化成像在cia中的应用。

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引用次数: 0

Imaging rheumatology: Emerging tools and expanding applications 风湿病影像学：新兴工具和扩展应用。

IF 4.8 2区医学 Q1 RHEUMATOLOGY

Best Practice & Research in Clinical Rheumatology

Pub Date : 2025-09-01 DOI: 10.1016/j.berh.2025.102088

Pamela Diaz P.D., MD, MSc , Gurjit S. Kaeley G.S.K., MBBS, MRCP, RhMSUS

引用次数: 0

New developments in ultrasound and MRI imaging of pediatric rheumatic diseases 风湿病的最佳实践：儿童风湿病超声和MRI成像的新发展。

IF 4.8 2区医学 Q1 RHEUMATOLOGY

Best Practice & Research in Clinical Rheumatology

Pub Date : 2025-09-01 DOI: 10.1016/j.berh.2025.102069

Johannes Roth , Clara Malattia

The evidence base for ultrasound and MRI imaging in pediatric rheumatic diseases continues to grow, enabling the routine clinical use of the two techniques that complement each other. Ultrasound imaging has a superior spatial resolution, the ability to assess several joints at the same time without sedation and provides very detailed assessments of important structures like the entheses. MRI has advantages in the assessment of osteochondral damage and certain anatomic regions like the temporomandibular joint (TMJ) and the axial skeleton. Significant progress in the standardization of image acquisition and quantification of findings allows the utilisation of both techniques as an outcome measure. This will improve the standard of care in pediatric rheumatic diseases where objective measurements of disease activity are urgently needed. Finally, the integration of artificial intelligence (AI) represents a groundbreaking development in the field and potential applications will be discussed.

超声和核磁共振成像在儿童风湿病中的证据基础不断增长，使这两种技术的常规临床应用能够相互补充。超声成像具有优越的空间分辨率，能够在没有镇静的情况下同时评估多个关节，并提供非常详细的重要结构评估，如关节。MRI在评估骨软骨损伤和某些解剖区域如颞下颌关节（TMJ）和中轴骨骼方面具有优势。在图像采集标准化和结果量化方面取得的重大进展允许将这两种技术用作结果衡量标准。这将提高儿科风湿病的护理标准，在那里迫切需要对疾病活动进行客观测量。最后，人工智能（AI）的集成代表了该领域的突破性发展，并将讨论潜在的应用。

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Advances in imaging techniques for Sjogren's disease 干燥病的影像技术进展。

IF 4.8 2区医学 Q1 RHEUMATOLOGY

Best Practice & Research in Clinical Rheumatology

Pub Date : 2025-09-01 DOI: 10.1016/j.berh.2025.102087

Baptiste Quéré , Sandrine Jousse-Joulin

Imaging of salivary glands (SG), particularly Salivary Glands Ultrasonography (SGUS) is increasingly used in patients with suspected Sjogren's disease (SD). SGUS is the first-line imaging modality. Numerous studies have highlighted this non-invasive, non-irradiating, and low-cost imaging modality. The OMERACT group has established a classification of SG structural damage based on B-mode findings, ranging from grade 0 (normal) to 3 (severe structural damage). SGUS abnormalities (≥ grade 2) have been reported in approximately 63 % of patients with SD. More recently, Hocevar et al. described a Doppler-based classification assessing SG parenchymal vascularization, graded from 0 (normal) to 3 (Doppler signals occupying the entire glandular surface) and could be used as a marker of disease activity and as a biomarker of response to therapy. Moreover, SGUS can be useful for looking for complications such as lymphoma. New ultrasound techniques are currently being developed, including elastography for assessing tissue stiffness, analysis of microvascularization using contrast-enhanced ultrasound with microbubbles, and analysis of minor salivary glands using the ultra-high frequency probe. The combination of several US modalities enhances both sensitivity and specificity of the technique, allowing for the development of a comprehensive multimodal imaging approach. Other imaging techniques can be performed for SD, such as MRI of the parotid glands, allowing analysis of the glandular parenchyma (“salt and pepper” appearance), and certain sequences (DWI-MR) should be performed when lymphoma or other tumors are suspected. 18-FDG PET-CT may be useful to detect systemic manifestations or complications in SD and new PET tracers are currently being developed.

唾液腺（SG）影像学检查，特别是唾液腺超声检查（SGUS）越来越多地用于疑似干燥病（SD）的患者。SGUS是一线成像方式。许多研究都强调了这种非侵入性、非辐照性和低成本的成像方式。OMERACT小组根据b模式的发现建立了SG结构损伤的分类，从0级（正常）到3级（严重结构损伤）。大约63%的SD患者报告了SGUS异常（≥2级）。最近，Hocevar等人描述了一种基于多普勒的分级，评估SG实质血管化，从0（正常）到3（多普勒信号占据整个腺体表面），可以用作疾病活度的标记和治疗反应的生物标记。此外，SGUS可用于寻找淋巴瘤等并发症。目前正在开发新的超声技术，包括用于评估组织刚度的弹性成像，使用微泡对比增强超声分析微血管化，以及使用超高频探头分析小唾液腺。几种US模式的结合增强了该技术的敏感性和特异性，从而允许开发一种全面的多模式成像方法。其他成像技术可用于SD，如腮腺的MRI，允许分析腺实质（“盐和胡椒”外观），当怀疑淋巴瘤或其他肿瘤时应进行某些序列（DWI-MR）。18-FDG PET- ct可用于检测SD的全身表现或并发症，目前正在开发新的PET示踪剂。

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