Current opinion in rheumatology最新文献_第10页

Myopathy in systemic sclerosis. 系统性硬化症中的肌病。

IF 5.2 2区医学

Current opinion in rheumatology Pub Date : 2023-11-01 Epub Date: 2023-08-22 DOI: 10.1097/BOR.0000000000000966

Caoilfhionn M Connolly, Julie J Paik

{"title":"Myopathy in systemic sclerosis.","authors":"Caoilfhionn M Connolly, Julie J Paik","doi":"10.1097/BOR.0000000000000966","DOIUrl":"10.1097/BOR.0000000000000966","url":null,"abstract":"Purpose of review: Systemic sclerosis associated myopathy (SSc-AM) is a complex, heterogenous disease that is associated with poor outcomes. SSc-AM lacks a clear definition, and continues to be poorly recognized. The purpose of this review is to provide a contemporary overview of the clinical, serological and pathophysiologic findings in SSc-AM to guide optimal recognition and management of this challenging disease manifestation.Recent findings: There have been several advances in diagnostic techniques to facilitate characterization of SSc-AM, including muscle MRI, in which findings were correlated to distinct histopathologic categories of muscle involvement in SSc, histopathologic findings of prominent fibrosis or inflammation on biopsy, and the identification of novel autoantibodies associated with SSc-AM, which may be associated with distinct clinical phenotypes. In one of the largest studies to date, 17% of a well phenotyped SSc cohort were found to have myopathy, which was an independent risk of death, even after adjusting for potential confounders, further highlighting the importance of timely recognistion and management of SSc-AM.Summary: There is increasing recognition of the importance of SSc-AM. Novel diagnostic tools provide the opportunity for more detailed insights into pathophysiologic mechanisms, which may facilitate the development of a rigorous consensus definition of SSc-AM.","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"341-348"},"PeriodicalIF":5.2,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10538402/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10178009","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

What is PFAPA syndrome? Genetic clues about the pathogenesis. 什么是PFAPA综合征？发病机制的遗传线索。

IF 5.2 2区医学

Current opinion in rheumatology Pub Date : 2023-11-01 Epub Date: 2023-07-17 DOI: 10.1097/BOR.0000000000000956

Kalpana Manthiram

{"title":"What is PFAPA syndrome? Genetic clues about the pathogenesis.","authors":"Kalpana Manthiram","doi":"10.1097/BOR.0000000000000956","DOIUrl":"10.1097/BOR.0000000000000956","url":null,"abstract":"Purpose of review: Periodic fever, aphthous stomatitis, pharyngitis, and cervical adenitis (PFAPA) syndrome is the most common periodic fever syndrome in childhood. Recent studies report genetic susceptibility variants for PFAPA syndrome and the efficacy of tonsillectomy in a broader cohort of patients with recurrent stereotypical fever. In this review, we highlight the findings of these studies and what they may reveal about the pathogenesis of PFAPA.Recent findings: Newly identified genetic susceptibility loci for PFAPA suggest that it is a complex genetic disorder linked to Behçet's disease and recurrent aphthous ulcers. Patients who have PFAPA with some features of Behçet's disease have been reported. Moreover, the efficacy of tonsillectomy has now been described in patients who do not meet the full diagnostic criteria for PFAPA, although the immunologic profile in the tonsils is different from those with PFAPA. Factors that predict response to tonsillectomy are also reported.Summary: These findings highlight the heterogeneous phenotypes that may be related to PFAPA due to common genetic susceptibility or response to therapy. These relationships raise questions about how to define PFAPA and highlight the importance of understanding of the genetic architecture of PFAPA and related diseases.","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"423-428"},"PeriodicalIF":5.2,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10538419/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9827769","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Exploring challenges in the management and treatment of inclusion body myositis. 探索包涵体肌炎的管理和治疗中的挑战。

IF 5.2 2区医学

Current opinion in rheumatology Pub Date : 2023-11-01 Epub Date: 2023-07-25 DOI: 10.1097/BOR.0000000000000958

Michael P Skolka, Elie Naddaf

{"title":"Exploring challenges in the management and treatment of inclusion body myositis.","authors":"Michael P Skolka, Elie Naddaf","doi":"10.1097/BOR.0000000000000958","DOIUrl":"10.1097/BOR.0000000000000958","url":null,"abstract":"Purpose of review: This review provides an overview of the management and treatment landscape of inclusion body myositis (IBM), while highlighting the current challenges and future directions.Recent findings: IBM is a slowly progressive myopathy that predominantly affects patients over the age of 40, leading to increased morbidity and mortality. Unfortunately, a definitive cure for IBM remains elusive. Various clinical trials targeting inflammatory and some of the noninflammatory pathways have failed. The search for effective disease-modifying treatments faces numerous hurdles including variability in presentation, diagnostic challenges, poor understanding of pathogenesis, scarcity of disease models, a lack of validated outcome measures, and challenges related to clinical trial design. Close monitoring of swallowing and respiratory function, adapting an exercise routine, and addressing mobility issues are the mainstay of management at this time.Summary: Addressing the obstacles encountered by patients with IBM and the medical community presents a multitude of challenges. Effectively surmounting these hurdles requires embracing cutting-edge research strategies aimed at enhancing the management and treatment of IBM, while elevating the quality of life for those affected.","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":"35 6","pages":"404-413"},"PeriodicalIF":5.2,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/ad/de/corhe-35-404.PMC10552844.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41194418","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Animal models in systemic sclerosis: an update. 系统性硬化症动物模型：最新进展。

IF 5.2 2区医学

Current opinion in rheumatology Pub Date : 2023-11-01 Epub Date: 2023-08-21 DOI: 10.1097/BOR.0000000000000967

Xiongjie Bi, Tingting Mills, Minghua Wu

{"title":"Animal models in systemic sclerosis: an update.","authors":"Xiongjie Bi, Tingting Mills, Minghua Wu","doi":"10.1097/BOR.0000000000000967","DOIUrl":"10.1097/BOR.0000000000000967","url":null,"abstract":"Purpose of review: Systemic sclerosis (SSc) is a multisystem autoimmune connective tissue disease characterized by early inflammation followed by excessive fibrosis in the skin and internal organs. Enhancing our comprehension of SSc pathogenesis is essential to develop effective therapeutic strategies. Animal models that mimic one or more aspects of SSc have been proven to be a valuable resource for investigating disease mechanisms. This review aims to provide an updated overview of the existing SSc animal models and the potentially relevant pathways to SSc pathogenesis.Recent findings: This review focuses on the most recently generated and investigated animal models, which delve into novel pathways beyond existing models or employ genetic technologies to gain a deeper understanding of SSc pathogenesis including activation of early type I interferon (IFN) signaling pathway, immune cell function and pulmonary artery hypertension (PAH).Summary: While no single animal model can fully replicate SSc, a combination of different models can offer valuable insights into the pathways involved in the onset and advancement of the SSc. These insights can prove animal models as a crutial preclinical tool for developing effective treatments for SSc.","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"364-370"},"PeriodicalIF":5.2,"publicationDate":"2023-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10553484/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"10414468","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Challenges in treating juvenile idiopathic arthritis. 治疗青少年特发性关节炎的挑战。

IF 5.1 2区医学

Current opinion in rheumatology Pub Date : 2023-09-01 DOI: 10.1097/BOR.0000000000000950

Marinka Twilt, Peter Stoustrup, Dax Rumsey

引用次数: 1

When to stop medication in juvenile idiopathic arthritis. 青少年特发性关节炎何时停止用药。

IF 5.2 2区医学

Current opinion in rheumatology Pub Date : 2023-09-01 Epub Date: 2023-05-03 DOI: 10.1097/BOR.0000000000000948

Dori Abel, Pamela F Weiss

{"title":"When to stop medication in juvenile idiopathic arthritis.","authors":"Dori Abel, Pamela F Weiss","doi":"10.1097/BOR.0000000000000948","DOIUrl":"10.1097/BOR.0000000000000948","url":null,"abstract":"Purpose of review: Disease-modifying antirheumatic drugs (DMARDs) have dramatically improved patient outcomes in juvenile idiopathic arthritis (JIA). However, these medications may also result in physical, psychologic, and economic burden, which must be balanced with risk of flare off treatment. Although some children remain in remission after medication discontinuation, evidence is sparse for if, when, and how medications should be de-escalated once achieving clinically inactive disease (CID). We review the data on medication discontinuation and the role of serologic and imaging biomarkers in JIA.Recent findings: The literature uniformly supports early biologic DMARD initiation, although the optimal timing and strategy for medication withdrawal in patients with sustained CID remains unclear. In this review, we present the current data on flare frequency and time to flare, clinical factors associated with flare, and recapture data for each JIA category. We also summarize the current knowledge on the role of imaging and serologic biomarkers in guiding these treatment decisions.Summary: JIA is a heterogenous disease for which prospective clinical trials are needed to address the question of when, how, and in whom to withdraw medication. Research investigating the roles of serologic and imaging biomarkers may help improve the ability to ascertain which children can successfully de-escalate medications.","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":"35 5","pages":"265-272"},"PeriodicalIF":5.2,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10526632/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9913609","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

引用次数: 0

Medium-vessel and large-vessel vasculitis in children. 儿童中、大血管炎。

IF 5.1 2区医学

Current opinion in rheumatology Pub Date : 2023-09-01 DOI: 10.1097/BOR.0000000000000955

Christiaan Scott, Raphaella Stander, Frank Phoya

{"title":"Medium-vessel and large-vessel vasculitis in children.","authors":"Christiaan Scott, Raphaella Stander, Frank Phoya","doi":"10.1097/BOR.0000000000000955","DOIUrl":"https://doi.org/10.1097/BOR.0000000000000955","url":null,"abstract":"Purpose of review: This article serves as an up-to-date examination of the latest findings in the field of paediatric large-vessel and medium-vessel vasculitis.Recent findings: Over the last 2 years and in the wake of SARS-CoV2 pandemic, a multitude of studies have increased our insight into these conditions. Although large-vessel and medium-vessel vasculitis are uncommon amongst children, they are a complex and multisystem with a constantly evolving landscape. Increasing numbers of reports from low-income and middle-income countries are shaping our understanding of the epidemiology of vasculitis in children. The influence of infectious disease and the microbiome are of particular interest in unravelling pathogenetic aspects. Improved understanding of the genetics and immunology offer opportunities for better diagnostic options and biomarkers of disease as well as targeted therapies.Summary: In this review, we address recent findings in epidemiology, pathophysiology, clinical findings, bio-markers, imaging and treatment that have the potential to offer better management solutions for these uncommon conditions.","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":"35 5","pages":"278-284"},"PeriodicalIF":5.1,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9918121","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

Update on autoinflammatory diseases. 自身炎症性疾病的最新进展。

IF 5.1 2区医学

Current opinion in rheumatology Pub Date : 2023-09-01 DOI: 10.1097/BOR.0000000000000953

Kosar Asna Ashari, Jonathan S Hausmann, Fatma Dedeoglu

{"title":"Update on autoinflammatory diseases.","authors":"Kosar Asna Ashari, Jonathan S Hausmann, Fatma Dedeoglu","doi":"10.1097/BOR.0000000000000953","DOIUrl":"https://doi.org/10.1097/BOR.0000000000000953","url":null,"abstract":"Purpose of review: Although the concept of systemic autoinflammatory diseases (SAIDs) is still very young, our knowledge about them is exponentially growing. In the current review, we aim to discuss novel SAIDs and autoinflammatory pathways discovered in the last couple of years.Recent findings: Advances in immunology and genetics have led to the discovery of new pathways involved in autoinflammation, as well as several new SAIDs, including retinal dystrophy, optic nerve edema, splenomegaly, anhidrosis, and migraine headache (ROSAH syndrome), vacuoles, E1 enzyme, X-linked autoinflammatory somatic (VEXAS) syndrome, TBK1 deficiency, NEMO deleted exon 5 autoinflammatory syndrome (NDAS), and disabling pansclerotic morphea. Progress in immunobiology and genetics has also brought forth novel treatments for SAIDs. Personalized medicine has made significant progress in areas such as cytokine-targeted therapies and gene therapies. However, much work remains, especially in measuring and improving the quality of life in patients with SAIDs.Summary: In the current review, we discuss the novelties in the world of SAIDs, including mechanistic pathways of autoinflammation, pathogenesis, and treatment. We hope this review helps rheumatologists to gain an updated understanding of SAIDs.","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":"35 5","pages":"285-292"},"PeriodicalIF":5.1,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9918117","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

Update on treatment responses and outcome measure development in chronic nonbacterial osteomyelitis. 慢性非细菌性骨髓炎治疗反应和结局指标进展的最新进展。

IF 5.1 2区医学

Current opinion in rheumatology Pub Date : 2023-09-01 DOI: 10.1097/BOR.0000000000000954

Shwetha Ramachandran, Yongdong Zhao, Polly J Ferguson

{"title":"Update on treatment responses and outcome measure development in chronic nonbacterial osteomyelitis.","authors":"Shwetha Ramachandran, Yongdong Zhao, Polly J Ferguson","doi":"10.1097/BOR.0000000000000954","DOIUrl":"https://doi.org/10.1097/BOR.0000000000000954","url":null,"abstract":"Purpose of review: To review recent trends in treatment and recent progress in developing outcome measures needed for chronic nonbacterial osteomyelitis (CNO) clinical trials.Recent findings: CNO is an autoinflammatory bone disease. In a minority of patients, the disease is genetically driven, and diagnosis can be made by DNA sequencing. However, for nonsyndromic CNO there is no diagnostic test. The number of children with CNO appears to be increasing and damage is common. Increases in CNO diagnosis is due to raised awareness, increased availability of whole-body magnetic resonance imaging and rising incidence. Treatment remains empiric and it is unclear which second line treatment is superior. Tumor necrosis factor inhibitors (TNFi) and bisphosphonates continue to be used as second line agents for nonsteroidal anti-inflammatory drugs (NSAID) refractory CNO; newer immune modulatory medications are used if this fails. Validated classification criteria, clinical outcome measures and imaging scoring standards are needed for successful clinical trials.Summary: Best treatment for NSAID refractory CNO remains unclear. Classification criteria, clinical outcomes measures and standardized imaging scoring have been developed or are near completion. This will facilitate robust clinical trials in CNO with the goal of having approved medications for this painful disease.","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":"35 5","pages":"255-264"},"PeriodicalIF":5.1,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9928215","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

New developments related to lung complications in pediatric rheumatic disease. 儿童风湿病肺部并发症的新进展

IF 5.1 2区医学

Current opinion in rheumatology Pub Date : 2023-09-01 DOI: 10.1097/BOR.0000000000000947

Shipra Rai, Grant S Schulert, Christopher Towe

{"title":"New developments related to lung complications in pediatric rheumatic disease.","authors":"Shipra Rai, Grant S Schulert, Christopher Towe","doi":"10.1097/BOR.0000000000000947","DOIUrl":"https://doi.org/10.1097/BOR.0000000000000947","url":null,"abstract":"Purpose of review: While substantial progress has been made understanding lung disease in adult patients with rheumatic disease, pediatric lung disease has not been well addressed. Several recent studies provide new insights into diagnosis, management and treatment of lung disease in children with rheumatic disease.Recent findings: Building on previous research, newly diagnosed patients may have abnormalities in pulmonary function tests and chest computed tomography imaging even when asymptomatic. New guidelines for screening for rheumatic-associated lung disease provide important recommendations for clinicians. New theories have been proposed about immunologic shifts leading to the development of lung disease in children with systemic juvenile idiopathic arthritis. Additionally, there are new antifibrotic agents that are being explored as treatments in pediatric patients with fibrotic lung diseases.Summary: Patients appear to have frequent lung function abnormalities while being clinically asymptomatic, emphasizing importance for rheumatologists to refer for pulmonary function tests and imaging at diagnosis. New advances are helping define optimal approaches to treatment of lung disease, including use of biologic agents and antifibrotic medicines for pediatric patients with rheumatologic diseases.","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":"35 5","pages":"273-277"},"PeriodicalIF":5.1,"publicationDate":"2023-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"9919415","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