Best Practice & Research in Clinical Rheumatology最新文献_第5页

Scleromyositis – The new frontier 硬化肌炎——新领域。

IF 4.8 2区医学

Best Practice & Research in Clinical Rheumatology Pub Date : 2026-05-01 Epub Date: 2026-03-06 DOI: 10.1016/j.berh.2026.102126

Marie Hudson , Yves Troyanov , Océane Landon-Cardinal

引用次数: 0

Recent advances in the heart and soul of primary cardiac involvement and cardiovascular disease in systemic sclerosis 系统性硬化症中原发性心脏受累和心血管疾病的心脏和灵魂研究进展。

IF 4.8 2区医学

Best Practice & Research in Clinical Rheumatology Pub Date : 2026-05-01 Epub Date: 2026-03-04 DOI: 10.1016/j.berh.2026.102124

Laura Ross , Cosimo Bruni

引用次数: 0

Cellular and molecular mechanisms of systemic sclerosis: Translation to targeted therapies and clinical practice 系统性硬化症的细胞和分子机制：转化为靶向治疗和临床实践。

IF 4.8 2区医学

Best Practice & Research in Clinical Rheumatology Pub Date : 2026-05-01 Epub Date: 2026-04-12 DOI: 10.1016/j.berh.2026.102137

Diane Documet, Sophia Ruser, Crystal Cheung, Monique Hinchcliff, Manvitha Nadella

引用次数: 0

Raynaud's phenomenon and digital ulceration in systemic sclerosis 系统性硬化症的雷诺现象与指端溃疡。

IF 4.8 2区医学

Best Practice & Research in Clinical Rheumatology Pub Date : 2026-05-01 Epub Date: 2026-02-27 DOI: 10.1016/j.berh.2026.102121

Amy Turnbull , John D. Pauling

{"title":"Raynaud's phenomenon and digital ulceration in systemic sclerosis","authors":"Amy Turnbull , John D. Pauling","doi":"10.1016/j.berh.2026.102121","DOIUrl":"10.1016/j.berh.2026.102121","url":null,"abstract":"<div><div>Systemic sclerosis (SSc) is a vascular disease. Immune-mediated endothelial injury leading to vasculopathy characterised by a progressive obliterative microangiopathy is a key aetiopathogenic driver of SSc. This typically manifests clinically as Raynaud's phenomenon (RP), which occurs in virtually all individuals with SSc and digital ulcers (DU), which develop in around half of patients at some stage in the disease course. The term RP encompasses a constellation of clinical features associated with digital vasospasm, typically in response to cold exposure. People with SSc-DU typically report more severe RP symptoms but it is an over-simplification to suggest that DU are simply a consequence of profound RP. The level of ischaemic tissue injury required to cause DU requires more protracted tissue hypoxia and there is a large body of work linking SSc-DU disease with progression of the obliterative microangiopathy and irreversible capillary loss typical of established SSc. The present chapter shall discuss the burden, aetiopathogenesis, assessment and management of SSc-RP and SSc-DU. We shall highlight practical considerations for the assessment and management of these digital vascular complications in routine clinical practice. The complexity of SSc-related digital vasculopathy is illustrated through examples from the clinic setting. The management of these complications shall focus on recently published clinical guidelines, highlighting nuances of management and the therapeutic rationale underpinning the use of unlicensed therapies for which the evidence base is sometimes scant.</div></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"40 2","pages":"Article 102121"},"PeriodicalIF":4.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147318827","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Juvenile systemic sclerosis 青少年系统性硬化症。

IF 4.8 2区医学

Best Practice & Research in Clinical Rheumatology Pub Date : 2026-05-01 Epub Date: 2026-02-03 DOI: 10.1016/j.berh.2026.102116

Ivan Foeldvari , Clare E. Pain

{"title":"Juvenile systemic sclerosis","authors":"Ivan Foeldvari , Clare E. Pain","doi":"10.1016/j.berh.2026.102116","DOIUrl":"10.1016/j.berh.2026.102116","url":null,"abstract":"<div><div>Juvenile systemic sclerosis (jSSc) is a rare multisystem autoimmune disease, representing 4–10 % of systemic sclerosis (SSc) cases, and causes significant morbidity during growth and development. While sharing features with adult-onset disease, jSSc differs in phenotype, with more frequent diffuse cutaneous involvement, overlap syndromes, distinct autoantibody profiles and different outcomes. Evidence to guide care remains limited because of disease rarity and the lack of paediatric trials.</div><div>This review summarises epidemiology, classification, clinical features and outcomes of jSSc, highlighting differences from adult disease. A structured approach to assessment is presented, emphasizing regular multisystem evaluation. Organ-specific assessment of skin, lung, cardiac, gastrointestinal, musculoskeletal, renal and vascular involvement, alongside patient- and parent-reported outcomes, is discussed. Management strategies are reviewed using paediatric consensus recommendations and extrapolated adult data, emphasizing specialist paediatric rheumatology care within multidisciplinary teams. Emerging therapies, including autologous haematopoietic stem cell transplantation, and issues of psychosocial impact and transition to adult care are addressed.</div></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"40 2","pages":"Article 102116"},"PeriodicalIF":4.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146120957","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Improving quality of care in systemic sclerosis 提高系统性硬化症的护理质量。

IF 4.8 2区医学

Best Practice & Research in Clinical Rheumatology Pub Date : 2026-05-01 Epub Date: 2026-01-31 DOI: 10.1016/j.berh.2026.102118

Aos Aboabat

引用次数: 0

Gastrointestinal involvement in systemic sclerosis: A spotlight on small bowel involvement, complications and management 系统性硬化症的胃肠道受累：聚焦小肠受累、并发症和管理。

IF 4.8 2区医学

Best Practice & Research in Clinical Rheumatology Pub Date : 2026-05-01 Epub Date: 2026-01-30 DOI: 10.1016/j.berh.2026.102117

Luis G. Alcala-Gonzalez , Carolina Malagelada , Zsuzsanna H. McMahan

{"title":"Gastrointestinal involvement in systemic sclerosis: A spotlight on small bowel involvement, complications and management","authors":"Luis G. Alcala-Gonzalez , Carolina Malagelada , Zsuzsanna H. McMahan","doi":"10.1016/j.berh.2026.102117","DOIUrl":"10.1016/j.berh.2026.102117","url":null,"abstract":"<div><div>Small-bowel involvement in systemic sclerosis is frequent but often underdiagnosed, largely due to the lack of standardized diagnostic protocols for its evaluation. Small bowel dysmotility manifests with symptoms such as abdominal pain, diarrhea, bloating, and distension, and complications including small intestinal bacterial overgrowth, reduced oral intake, malnutrition, and, in severe cases, intestinal pseudo-obstruction. These complications are associated with significant morbidity, dependence on parenteral nutrition, and poor prognosis. In this review, we summarize the current evidence on the pathogenesis, diagnosis, and management of small-bowel involvement, with a focus on advances in understanding autoimmune neuromuscular mechanisms and their clinical and serological correlates. We discuss diagnostic approaches and propose a structured, multidisciplinary treatment strategy integrating nutritional optimization, targeted prokinetic and antibiotic therapy, and management of acute pseudo-obstructive crises. Finally, we highlight future directions, including the development of biomarkers, advanced motility assessments, and novel therapeutic approaches aimed at earlier diagnosis and personalized care.</div></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"40 2","pages":"Article 102117"},"PeriodicalIF":4.8,"publicationDate":"2026-05-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146097520","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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

Complexity to clarity: Advancing the science and care of systemic sclerosis 从复杂到清晰：推进系统性硬化症的科学和治疗。

IF 4.8 2区医学

Best Practice & Research in Clinical Rheumatology Pub Date : 2026-05-01 Epub Date: 2026-05-06 DOI: 10.1016/j.berh.2026.102154

Sindhu R. Johnson MD PhD

引用次数: 0

CAR T-cell therapy and indications for pediatric rheumatology CAR - t细胞治疗和儿童风湿病的适应症：应用、风险和考虑的结构化概述。

IF 4.8 2区医学

Best Practice & Research in Clinical Rheumatology Pub Date : 2026-03-01 Epub Date: 2026-04-02 DOI: 10.1016/j.berh.2026.102143

Kali Chiriboga , Pooja N. Patel , Hermine I. Brunner

引用次数: 0

Axial spondyloarthritis: Moving beyond cytokine based therapies to cellular targets and advanced therapeutics 轴性脊柱炎：超越细胞因子治疗到细胞靶点和先进治疗。

IF 4.8 2区医学

Best Practice & Research in Clinical Rheumatology Pub Date : 2026-03-01 Epub Date: 2026-05-12 DOI: 10.1016/j.berh.2026.102155

Surbhi Patel , Rahul Patel , Mohamad Bittar , Atul Deodhar

{"title":"Axial spondyloarthritis: Moving beyond cytokine based therapies to cellular targets and advanced therapeutics","authors":"Surbhi Patel , Rahul Patel , Mohamad Bittar , Atul Deodhar","doi":"10.1016/j.berh.2026.102155","DOIUrl":"10.1016/j.berh.2026.102155","url":null,"abstract":"<div><div>Rheumatology as a specialty is moving past cytokine inhibitors which have dominated the therapeutic landscape since the turn of the century, with novel cell-targeting therapies emerging to treat immune-mediated multisystem inflammatory diseases such as systemic lupus, systemic sclerosis, small vessel vasculitides, and inflammatory myositis. Like many inflammatory arthritides, axial spondyloarthritis (axSpA) therapeutics has been limited to either inhibitors of TNF and IL-17, or blocking intracellular signaling of cytokines through Janus Kinase inhibitors. This systematic review chronicles the clinical progress made in the field of selective T cell depleting agents, the potential for novel cellular targeting therapies using Chimeric Antigen Receptor - T <strong>(</strong>CAR-T) therapy, as well the challenges posed by this approach for treatment of axSpA. A review of the MEDLINE, OVID and clinicaltrials.gov databases was performed which revealed no ongoing or completed open label or completed registered clinical trials. However recent data have highlighted potential for selective CD8<sup>+</sup> T cell targets with proof of concept using a monoclonal antibody against TRBV9+ CD8<sup>+</sup> T cells from a published case study and one randomized placebo-controlled trial, as well as other emerging therapeutics that are shifting focus from cytokine-based therapeutics to T cell targeting agents. In this review, we identified emerging therapeutic platforms of cellular immunotherapy, potential therapeutic targets, and discuss potential adverse effects related to cell-targeting therapies. While at present there is a dearth of clinical data on cellular targeting treatment options in axSpA, ongoing investigations into the pathogenesis of axSpA and identification of new molecular targets may help facilitate opportunities to develop CAR-T and other cell-based therapies for treatment of axSpA.</div></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"40 1","pages":"Article 102155"},"PeriodicalIF":4.8,"publicationDate":"2026-03-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147876402","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"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