Pub Date : 2026-02-03DOI: 10.1016/j.berh.2026.102116
Ivan Foeldvari, Clare E Pain
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.
{"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}
Pub Date : 2026-01-31DOI: 10.1016/j.berh.2026.102118
Aos Aboabat
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.
{"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}
Pub Date : 2026-01-30DOI: 10.1016/j.berh.2026.102117
Luis G Alcala-Gonzalez, Carolina Malagelada, Zsuzsanna H McMahan
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.
{"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}
Pub Date : 2025-12-01DOI: 10.1016/j.berh.2025.102106
Tania Gudu , Mert Oztas , Elena Nikiphorou
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.
{"title":"Contemporary approaches to the management of rheumatoid arthritis: precision and progress","authors":"Tania Gudu , Mert Oztas , 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}
Pub Date : 2025-12-01DOI: 10.1016/j.berh.2025.102105
Marta Dzhus , Walter P. Maksymowych
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.
{"title":"Novel therapies for axial spondyloarthritis and future directions","authors":"Marta Dzhus , 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}
Pub Date : 2025-12-01DOI: 10.1016/j.berh.2025.102100
Charles Inderjeeth, Diren Che Inderjeeth
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.
{"title":"Novel therapies in osteoporosis – Clinical update – 2025","authors":"Charles Inderjeeth, 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}
Pub Date : 2025-12-01DOI: 10.1016/j.berh.2025.102101
Yoshiya Tanaka
Systemic lupus erythematosus (SLE) is an autoimmune disease that damages multiple organs. Glucocorticoids (GCs) have been a mainstay of the treatment of SLE, but it is strongly recommended to minimize GCs usage due to the toxicity of long-term use. Currently, development of molecular-targeted therapies based on pathological mechanisms is underway. Activation of B cells through T-B cell interaction play a central role in the pathogenesis, and treatments targeting B cells and co-stimulatory molecules are expected. In addition, many disease susceptibility genes are mediated in signaling by the innate immune mechanisms, such as dendritic cells involvement and cytokines production that stimulates acquired immunity, as well as kinases of intracellular signaling molecules that are described as targets. Furthermore, adoptive transfer of T cells engineered to target CD19 antigen by gene transfer of chimeric antigen receptor and by T cell engagers that recruit T cells and induce B cell cytotoxicity gather attention.
{"title":"Novel therapies in treatments of SLE","authors":"Yoshiya Tanaka","doi":"10.1016/j.berh.2025.102101","DOIUrl":"10.1016/j.berh.2025.102101","url":null,"abstract":"<div><div>Systemic lupus erythematosus (SLE) is an autoimmune disease that damages multiple organs. Glucocorticoids (GCs) have been a mainstay of the treatment of SLE, but it is strongly recommended to minimize GCs usage due to the toxicity of long-term use. Currently, development of molecular-targeted therapies based on pathological mechanisms is underway. Activation of B cells through T-B cell interaction play a central role in the pathogenesis, and treatments targeting B cells and co-stimulatory molecules are expected. In addition, many disease susceptibility genes are mediated in signaling by the innate immune mechanisms, such as dendritic cells involvement and cytokines production that stimulates acquired immunity, as well as kinases of intracellular signaling molecules that are described as targets. Furthermore, adoptive transfer of T cells engineered to target CD19 antigen by gene transfer of chimeric antigen receptor and by T cell engagers that recruit T cells and induce B cell cytotoxicity gather attention.</div></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"39 4","pages":"Article 102101"},"PeriodicalIF":4.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145253228","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}
Pub Date : 2025-12-01DOI: 10.1016/j.berh.2025.102102
Graeme Jones
Osteoarthritis is major and growing public health problem. Major advances have occurred in terms of understanding the pathogenesis of the disease primarily due to advances in imaging. Both systemic and local factors are involved. Some of these are modifiable making them attractive targets for therapy and the development of precision medicine in osteoarthritis. A growing number of these trials selecting subgroups of osteoarthritis with specific imaging features have been completed with mixed results. In many cases, the interventional studies have not replicated the results of the basic science and human observational studies and the majority of trials have been negative with a few notable exceptions. There is an urgent need for greater choice of therapies in this condition.
{"title":"What's new in osteoarthritis?","authors":"Graeme Jones","doi":"10.1016/j.berh.2025.102102","DOIUrl":"10.1016/j.berh.2025.102102","url":null,"abstract":"<div><div>Osteoarthritis is major and growing public health problem. Major advances have occurred in terms of understanding the pathogenesis of the disease primarily due to advances in imaging. Both systemic and local factors are involved. Some of these are modifiable making them attractive targets for therapy and the development of precision medicine in osteoarthritis. A growing number of these trials selecting subgroups of osteoarthritis with specific imaging features have been completed with mixed results. In many cases, the interventional studies have not replicated the results of the basic science and human observational studies and the majority of trials have been negative with a few notable exceptions. There is an urgent need for greater choice of therapies in this condition.</div></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"39 4","pages":"Article 102102"},"PeriodicalIF":4.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145214362","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}
Pub Date : 2025-12-01DOI: 10.1016/j.berh.2025.102108
Arjun Mahajan , Alex Tinianow , Guy Katz
Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder that can affect many organ systems. This chapter provides a comprehensive overview of current knowledge regarding IgG4-RD, addressing key questions about disease mechanisms, diagnostic approaches, and therapeutic strategies. We examine the diverse clinical manifestations ranging from glandular enlargement to life-threatening vascular involvement, emphasizing the importance of recognizing both inflammatory and fibrotic disease phenotypes. Diagnostic approaches integrate clinical symptoms, imaging findings, histopathological features, and serological markers while excluding mimicking conditions. Treatment strategies have evolved significantly with the introduction of B cell depletion therapy, particularly inebilizumab. Current treatment paradigms must prioritize glucocorticoid-sparing approaches, though maintenance therapy considerations remain complex. Critical knowledge gaps persist regarding optimal biomarkers for disease activity, standardized remission criteria, and the precise etiopathogenesis of IgG4-RD. Despite therapeutic advances, challenges remain in balancing effective disease control with minimizing the harms of long-term immunosuppression, emphasizing the need for continued research into targeted therapies.
{"title":"IgG4-Related disease: From diagnosis to remission","authors":"Arjun Mahajan , Alex Tinianow , Guy Katz","doi":"10.1016/j.berh.2025.102108","DOIUrl":"10.1016/j.berh.2025.102108","url":null,"abstract":"<div><div>Immunoglobulin G4-related disease (IgG4-RD) is a systemic fibroinflammatory disorder that can affect many organ systems. This chapter provides a comprehensive overview of current knowledge regarding IgG4-RD, addressing key questions about disease mechanisms, diagnostic approaches, and therapeutic strategies. We examine the diverse clinical manifestations ranging from glandular enlargement to life-threatening vascular involvement, emphasizing the importance of recognizing both inflammatory and fibrotic disease phenotypes. Diagnostic approaches integrate clinical symptoms, imaging findings, histopathological features, and serological markers while excluding mimicking conditions. Treatment strategies have evolved significantly with the introduction of B cell depletion therapy, particularly inebilizumab. Current treatment paradigms must prioritize glucocorticoid-sparing approaches, though maintenance therapy considerations remain complex. Critical knowledge gaps persist regarding optimal biomarkers for disease activity, standardized remission criteria, and the precise etiopathogenesis of IgG4-RD. Despite therapeutic advances, challenges remain in balancing effective disease control with minimizing the harms of long-term immunosuppression, emphasizing the need for continued research into targeted therapies.</div></div>","PeriodicalId":50983,"journal":{"name":"Best Practice & Research in Clinical Rheumatology","volume":"39 4","pages":"Article 102108"},"PeriodicalIF":4.8,"publicationDate":"2025-12-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"145530788","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}
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