Best Practice & Research in Clinical Rheumatology最新文献

{"title":"Raynaud's phenomenon and digital ulceration in systemic sclerosis.","authors":"Amy Turnbull, John D Pauling","doi":"10.1016/j.berh.2026.102121","DOIUrl":"https://doi.org/10.1016/j.berh.2026.102121","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":" ","pages":"102121"},"PeriodicalIF":4.8,"publicationDate":"2026-02-26","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}

{"title":"The challenges in managing Psoriatic Arthritis in resource poor settings.","authors":"Jan René Nkeck, Adewale O Adebajo, Ajesh B Maharaj","doi":"10.1016/j.berh.2026.102123","DOIUrl":"https://doi.org/10.1016/j.berh.2026.102123","url":null,"abstract":"<p><strong>Background: </strong>Psoriatic arthritis (PsA) is a systemic autoimmune and auto-inflammatory disease associated with significant morbidity, increased mortality, and substantial socioeconomic burden globally. While therapeutic advances have improved outcomes, their implementation in resource-limited settings faces profound challenges.</p><p><strong>Methods: </strong>This narrative review synthesises findings from relevant publications identified via PubMed, Medline, Google Scholar and Embase, focusing on the management of PsA in resource-limited settings.</p><p><strong>Results: </strong>We identified four critical challenge domains. Epidemiological: a severe lack of robust population-based data on prevalence, incidence, and phenotype in resource-limited settings hinders public health planning.</p><p><strong>Diagnostic: </strong>significant delays occur due to a scarcity of specialists, limited access to diagnostic tools, and unclear patient pathways, leading to missed therapeutic opportunities and increased structural damage. Therapeutic: access to conventional and advanced biologic/targeted therapies is severely restricted by high costs, limited availability, and insufficient health coverage. This is compounded by a lack of adapted treatment guidelines and multidisciplinary care infrastructure.</p><p><strong>Research: </strong>minimal local research capacity, few clinical trials, and limited funding stifle innovation and context-specific solution development.</p><p><strong>Conclusion: </strong>The effective management of PsA in resource-limited settings requires a multifaceted strategy. Key priorities include: strengthening health systems through clear care pathways; task-shifting and enhanced training for primary care providers; developing national registries and context-adapted guidelines; advocating for improved drug access and affordability; and fostering local research capacity. Addressing these challenges is essential to mitigate health inequities and improve outcomes for PsA patients in resource-limited settings.</p>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":" ","pages":"102123"},"PeriodicalIF":4.8,"publicationDate":"2026-02-24","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"147286043","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}

{"title":"Oral and maxillofacial manifestations of systemic sclerosis.","authors":"Saeed Almurait, Hanan Alqahtani, Savithiri Ratnapalan, Sindhu R Johnson","doi":"10.1016/j.berh.2026.102119","DOIUrl":"https://doi.org/10.1016/j.berh.2026.102119","url":null,"abstract":"<p><p>Systemic sclerosis (SSc) is a systemic autoimmune rheumatic disease characterized by fibrosis, microvascular damage, and immune dysregulation. Although its cutaneous and systemic features have been extensively delineated, its oral and maxillofacial features are not well studied. Microstomia is one of the most incapacitating oral symptoms in SSc, hampering oral hygiene, and dental treatment. Xerostomia leads to increased caries susceptibility, whereas periodontal disease and vascular insufficiency hasten tissue breakdown, leading to attachment loss and bone resorption. Temporomandibular joint involvement undermines jaw mobility, leading to discomfort, and a reduction in food intake. Scleroglossus and frenum fibrosis impair clear speech. Orofacial exercises can strengthen mouth opening. Dental implants and regenerative procedures appear promising but need to be undertaken with careful patient selection. This review synthesizes current evidence on the prevalence, pathogenesis, and clinical management of SSc oral and maxillofacial manifestations and their complications.</p>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":" ","pages":"102119"},"PeriodicalIF":4.8,"publicationDate":"2026-02-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146203676","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}

{"title":"Juvenile systemic sclerosis.","authors":"Ivan Foeldvari, Clare E Pain","doi":"10.1016/j.berh.2026.102116","DOIUrl":"https://doi.org/10.1016/j.berh.2026.102116","url":null,"abstract":"<p><p>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. 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.</p>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":" ","pages":"102116"},"PeriodicalIF":4.8,"publicationDate":"2026-02-03","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}

{"title":"Improving quality of care in systemic sclerosis.","authors":"Aos Aboabat","doi":"10.1016/j.berh.2026.102118","DOIUrl":"https://doi.org/10.1016/j.berh.2026.102118","url":null,"abstract":"<p><p>Systemic sclerosis is a complex multisystem autoimmune disease associated with early mortality, disability, and impaired quality of life. This chapter reviews the quality of care delivered to people with systemic sclerosis and addresses three questions: how current practice aligns with guideline-based care, where the major gaps occur across the disease course, and which quality improvement strategies show promise. Studies from cohorts, registries, and patient surveys identify incomplete cardiopulmonary screening, underuse of rehabilitation and preventive care, and fragmented multispecialty management. Early quality improvement efforts, including multidisciplinary pathways, electronic health record tools, and use of disease specific quality indicators, improve adherence to recommended processes but remain small in scale and focused on intermediate outcomes. We highlight priorities for future work, including use of implementation science, digital infrastructure, and patient reported outcomes to build learning health systems that reduce unwarranted variation in systemic sclerosis care.</p>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":" ","pages":"102118"},"PeriodicalIF":4.8,"publicationDate":"2026-01-31","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"146101035","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}

{"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":"https://doi.org/10.1016/j.berh.2026.102117","url":null,"abstract":"<p><p>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.</p>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":" ","pages":"102117"},"PeriodicalIF":4.8,"publicationDate":"2026-01-30","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}

{"title":"Novel Therapy","authors":"Mitsumasa Kishimoto MD, PhD ,&nbsp;Peter Nash MD","doi":"10.1016/j.berh.2025.102103","DOIUrl":"10.1016/j.berh.2025.102103","url":null,"abstract":"","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"39 4","pages":"Article 102103"},"PeriodicalIF":4.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145180235","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}

{"title":"Contemporary approaches to the management of rheumatoid arthritis: precision and progress","authors":"Tania Gudu ,&nbsp;Mert Oztas ,&nbsp;Elena Nikiphorou","doi":"10.1016/j.berh.2025.102106","DOIUrl":"10.1016/j.berh.2025.102106","url":null,"abstract":"<div><div>Rheumatoid arthritis (RA) is a chronic autoimmune disease affecting synovial joints and extra-articular organs. While conventional synthetic disease-modifying antirheumatic drugs (csDMARDs) like methotrexate remain the cornerstone of therapy, nearly half of patients demonstrate inadequate response to monotherapy. This review provides a comprehensive overview of the evolving therapeutic landscape in RA, covering standard therapies, novel treatments, and precision medicine approaches. The treatment landscape has dramatically expanded with biologic DMARDs targeting TNF-α, IL-6, B and T cells, and more recently, targeted synthetic DMARDs such as the Janus kinase inhibitors (JAKi). Emerging therapies are discussed, including innovative cell-based approaches, that will likely continue to revolutionize the treatment o. Furthermore, treatment failure, in the context of difficult-to-treat disease and factors associated with this, are addressed. We explore biomarker-driven treatment selection utilizing autoantibodies, imaging, and synovial tissue analysis and address key challenges around drug safety concerns, managing comorbidities and difficult-to-treat RA. Finally, this article concludes with reflections on future directions and the role of machine learning, multi-omics technologies, while maintaining the focus on patient-centered approaches to care.</div></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"39 4","pages":"Article 102106"},"PeriodicalIF":4.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145214343","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}

{"title":"Novel therapies for axial spondyloarthritis and future directions","authors":"Marta Dzhus ,&nbsp;Walter P. Maksymowych","doi":"10.1016/j.berh.2025.102105","DOIUrl":"10.1016/j.berh.2025.102105","url":null,"abstract":"<div><div>This update explores emerging therapeutic strategies aimed at novel targets implicated in the pathogenesis of axSpA. Recent clinical trials of bimekizumab, a monoclonal antibody targeting both IL-17A and IL-17F, and janus-kinase inhibitors have demonstrated significant and sustained improvements in clinical and imaging outcomes, with a favorable safety profile and reduced rates of uveitis. Investigational agents targeting GM-CSF and MK2 have not demonstrated efficacy, but the targeting of autoreactive T cell clonotypes shared among individuals with axSpA using depleting antibodies to the variable gene segment 9 of the T cell receptor beta chain appears promising. Preclinical investigation has focused on cytokines, such as macrophage inflammatory protein, and kinases, such as mammalian target of rapamycin and phosphoinositide 3-kinase, and transcriptional factors, such as retinoic acid receptor-related orphan receptor-yt that regulate expression of IL-17A and -F cytokines. Several advances in therapeutic technologies also hold promise for more effective therapeutics based on current targets.</div></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"39 4","pages":"Article 102105"},"PeriodicalIF":4.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145276190","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}

{"title":"Novel therapies in osteoporosis – Clinical update – 2025","authors":"Charles Inderjeeth,&nbsp;Diren Che Inderjeeth","doi":"10.1016/j.berh.2025.102100","DOIUrl":"10.1016/j.berh.2025.102100","url":null,"abstract":"<div><div>Rheumatological patients are at high risk of osteoporosis and fracture due to disease, treatments, comorbidity and physical and functional considerations. Treating osteoporosis optimally is paramount. Osteoporosis management is evolving rapidly beyond traditional therapies. This 2025 review examines available traditional therapies and novel pharmacological approaches developed. Key questions addressed include the mechanisms, efficacy, and safety of agents like the sclerostin inhibitor romosozumab, Parathyroid hormone targeted agents, cytokine inhibitors and the status of therapies targeting cathepsin K. We evaluate the growing importance of combined and sequential treatment strategies, particularly initiating potent anabolic or dual-action therapies followed by antiresorptives for high-risk patients. Furthermore, the review explores emerging therapeutic targets such as modulators of the Wnt pathway, inflammation, and bone cell metabolism, alongside advancements in drug delivery, gene therapy, and non-pharmacological interventions. Clinical implications for patient selection, monitoring, and navigating the expanding treatment landscape are discussed, highlighting future directions towards personalized osteoporosis care.</div></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"39 4","pages":"Article 102100"},"PeriodicalIF":4.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145151689","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}