{"title":"Interstitial lung disease and myositis.","authors":"Takahisa Gono, Masataka Kuwana","doi":"10.1097/BOR.0000000000001037","DOIUrl":"10.1097/BOR.0000000000001037","url":null,"abstract":"<p><strong>Purpose of review: </strong>In patients with myositis, interstitial lung disease (ILD) is one of the major causes of morbidity and mortality. Given the limited evidence, there is an urgent need to refine the treatment for myositis-ILD. This review aims to highlight recent updates on the management of myositis-associated ILD, focusing on screening, risk stratification, and treatment.</p><p><strong>Recent findings: </strong>Asian race and/or residence, dermatomyositis, mechanic's hand, antisynthetase antibodies, and antimelanoma differentiation-associated gene 5 antibodies are risk factors for ILD development. Patients with such risk factors should be screened for ILD using high-resolution computed tomography. Various prediction models for mortality or rapidly progressive ILD (RP-ILD) in patients with myositis-ILD have been proposed, but validation of these models in multiple independent studies is required. Academic societies in Japan, the United Kingdom, and the United States have proposed tentative treatment algorithms for myositis-ILD on the basis of the presence or absence of RP-ILD.</p><p><strong>Summary: </strong>Knowledge on myositis-ILD risk stratification, potentially useful for personalized management approaches in clinical practice, is accumulating. However, further global joint efforts are necessary to build a strong evidence base for consensus algorithms for myositis-ILD.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"466-472"},"PeriodicalIF":5.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141916351","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}
Rochelle L Castillo, Kimberly Hashemi, Elizabeth Rainone, Katharina S Shaw, Ruth Ann Vleugels
{"title":"From traditional to targeted: the changing trajectory of therapies in dermatomyositis.","authors":"Rochelle L Castillo, Kimberly Hashemi, Elizabeth Rainone, Katharina S Shaw, Ruth Ann Vleugels","doi":"10.1097/BOR.0000000000001041","DOIUrl":"10.1097/BOR.0000000000001041","url":null,"abstract":"<p><strong>Purpose of review: </strong>New breakthroughs in our understanding of dermatomyositis (DM) have spawned the recent development of novel agents that specifically target key drivers in DM immunopathogenesis. This review aims to provide a comprehensive overview of new and forthcoming therapies for DM and to highlight their mechanisms of action, best evidence to date, and potential impact on disease management.</p><p><strong>Recent findings: </strong>Strategies that either counteract dysregulated interferon signaling [via the inhibition of interferon β, the type I interferon receptor subunit 1 (IFNAR1), or janus kinase (JAK)-signal transducer and activator of transcription (STAT) transduction] or induce durable autoreactive B cell depletion through chimeric antigen receptor (CAR) T-cell therapy appear to hold the most promise for sustained remission in DM.</p><p><strong>Summary: </strong>The trajectory of DM treatments is rapidly evolving, fueled by the unparalleled insights provided by multiomic studies and big data analysis pipelines. Targeted therapies that maximize both efficacy and safety have the potential to complement or replace traditional immunosuppressives and revolutionize the approach to the management of DM.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"438-444"},"PeriodicalIF":5.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141975266","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}
Zsuzsanna McMahan, John Pandolfino, Harris Perlman, Francesco Del Galdo, Monique Hinchcliff
{"title":"Gastrointestinal disease in systemic sclerosis: the neglected organ system?","authors":"Zsuzsanna McMahan, John Pandolfino, Harris Perlman, Francesco Del Galdo, Monique Hinchcliff","doi":"10.1097/BOR.0000000000001052","DOIUrl":"10.1097/BOR.0000000000001052","url":null,"abstract":"<p><strong>Purpose of review: </strong>Identifying outcomes and clinical trial endpoints enabled the discovery of new inflammatory bowel disease (IBD) treatments. Herein, we describe efforts to advance the study of gastrointestinal (GI) manifestations in systemic sclerosis (SSc).</p><p><strong>Recent findings: </strong>Insights into the scope of the problem, as well as advancements in the measurement and treatment of SSc-GI, are underway. Proposed SSc esophageal endophenotypes are now defined, risk stratification methods are growing, and imaging and functional studies are now employed to guide therapeutic interventions. Additional progress is being made in characterizing the gut microbiome in patients with SSc. Research into the role of the immune response in the pathogenesis of SSc-GI disease is also ongoing, evolving simultaneously with the development of methods to facilitate data collection with real-time capture of diet, exercise, and medication data.</p><p><strong>Summary: </strong>Multidisciplinary teams are working to deepen our understanding of SSc-GI disease pathogenesis, to identify biomarkers for risk stratification and the assessment of disease activity, and to develop and validate outcomes and clinical trial endpoints to pave the way toward effective therapy for SSc-GI disease.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"374-378"},"PeriodicalIF":5.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11588520/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079590","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}
{"title":"Antimelanoma differentiation antigen 5-positive dermatomyositis: an update.","authors":"Xin Lu, Qinglin Peng, Guochun Wang","doi":"10.1097/BOR.0000000000001034","DOIUrl":"10.1097/BOR.0000000000001034","url":null,"abstract":"<p><strong>Purpose of review: </strong>Antimelanoma differentiation antigen 5-dermatomyositis (MDA5-DM) is a complex and serious systemic autoimmune disease that primarily affects the skin and lungs. In this review, we aimed to provide new insights into the clinical features, pathogenesis, and practical management approach for this disease.</p><p><strong>Recent findings: </strong>Although lung lesions are prominent in most patients with MDA5-DM, they are now recognized as heterogeneous diseases. Peripheral blood lymphocyte count can serve as a simple and reliable laboratory parameter for categorizing MDA5-DM into three subgroups: mild, medium, and severe. Recent studies have implicated viral infection, genetic factors, autoimmunity against MDA5, multiple immune cells, and interferons as significant contributors to MDA5-DM pathogenesis. In addition to traditional treatments with glucocorticoids and immunosuppressants, many new approaches, including new biologics and targeted agents, have been explored. Additionally, infection is a common complication of MDA5-DM, and prophylaxis or treatment of the infection is as important as treating the primary disease.</p><p><strong>Summary: </strong>Knowledge of clinical characteristics and pathogenesis of MDA5-DM has grown in recent years. Although many new therapeutic approaches have been explored, further studies are required to confirm their efficacy.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"459-465"},"PeriodicalIF":5.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141616078","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":"Treatment of interstitial lung disease in systemic sclerosis: guidelines and new clinical trial results.","authors":"Sindhu R Johnson, Elana J Bernstein","doi":"10.1097/BOR.0000000000001049","DOIUrl":"10.1097/BOR.0000000000001049","url":null,"abstract":"<p><strong>Purpose of review: </strong>Interstitial lung disease (ILD) is the leading cause of death in patients with systemic sclerosis-associated interstitial lung disease (SSc-ILD). The American College of Rheumatology (ACR), in conjunction with the American College of Chest Physicians (CHEST), recently published clinical practice guidelines for the treatment of adults with systemic autoimmune rheumatic disease-associated ILD, including SSc-ILD. Herein, we summarize evidence from randomized trials evaluating the safety and efficacy of pharmacologic therapies for the treatment of SSc-ILD.</p><p><strong>Recent findings: </strong>In this review, we present findings from recent randomized controlled trials in SSc-ILD. The pharmacologic therapies discussed include immunosuppressive medications (mycophenolate, cyclophosphamide, rituximab, and tocilizumab) and antifibrotic medications (nintedanib and pirfenidone).</p><p><strong>Summary: </strong>Randomized trials provide an evidence base for the SSc-ILD treatment recommendations put forth in the ACR/CHEST Guidelines for the treatment of ILD in people with systemic autoimmune rheumatic diseases. These guidelines will help inform clinical practice and highlight areas in which further research is needed.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"420-426"},"PeriodicalIF":5.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11588535/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142079591","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}
{"title":"Heart involvement in systemic sclerosis: emerging concepts.","authors":"Jessica L Fairley, Laura Ross, Mandana Nikpour","doi":"10.1097/BOR.0000000000001038","DOIUrl":"10.1097/BOR.0000000000001038","url":null,"abstract":"<p><strong>Purpose of review: </strong>Systemic sclerosis (SSc)-associated heart involvement (SHI) is a significant cause of both morbidity and mortality in individuals with SSc. SHI can take many different forms, and likely is a spectrum of fibroinflammatory cardiac disease. Presenting features include arrhythmia, ventricular systolic or diastolic dysfunction, pericardial disease, and exercise intolerance. Risk of sudden cardiac death in SSc is likely 10-30-fold greater than general population estimates. In this review, we explore what is known about the pathogenesis of SHI, its prevention and management, and discuss available strategies for screening for SHI in light of new recommendations for the routine screening of SHI in all SSc patients.</p><p><strong>Recent findings: </strong>We describe the spectrum, clinical features, and pathogenesis of SHI. Furthermore, we review the new recommendations for screening for SHI in individuals with SSc.</p><p><strong>Summary: </strong>There is a large, under-recognized burden of SHI in people living with SSc, which likely contributes to the significant increase in sudden cardiac death observed in SSc. However, a broad-based screening approach, including asymptomatic, low-risk patients should be viewed with caution given the lack of evidence-based treatments and interventions for SHI particularly in this group.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"393-400"},"PeriodicalIF":5.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141906149","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}
Fionnuala K McMorrow, Natalie Anwyll, Sarah L Tansley
{"title":"Autoantibody testing in myositis: an update.","authors":"Fionnuala K McMorrow, Natalie Anwyll, Sarah L Tansley","doi":"10.1097/BOR.0000000000001039","DOIUrl":"10.1097/BOR.0000000000001039","url":null,"abstract":"<p><strong>Purpose of review: </strong>This review aims to provide an update on myositis autoantibody testing strategies. We have focussed on the reliability and usefulness of different myositis autoantibody detection methods, including commonly used solid phase immunoassays and newer discovery techniques.</p><p><strong>Recent findings: </strong>Several studies have highlighted the limitations of currently available immunoassays, particularly when used in populations with low pretest probability and without supporting clinical evidence. While many autoantibodies, such as anti-Jo1, are detected with high sensitivity and specificity, the low incidence of myositis autoantibodies in tested populations reduces their positive predictive value. The low sensitivity of line immunoassays to detect key myositis autoantibodies, including anti-TIF1γ and rarer antisynthetase autoantibodies, is a concern.</p><p><strong>Summary: </strong>Myositis autoantibodies are widely accepted as important clinical tools, and hence, there is a significant demand for reliable, accessible, and affordable detection methods. False positives and negative results have the potential to impact on patient care, particularly for malignancy and lung disease associated autoantibodies. Increased availability of myositis autoantibody testing has led to a rise in requests from a broader range of clinicians. It is critically important that clinicians are aware of specific limitations of tests and interpret results in the context of clinical findings.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"481-487"},"PeriodicalIF":5.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141999525","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":"Current approach to muscle imaging in myositis.","authors":"Didem Saygin, Jemima Albayda","doi":"10.1097/BOR.0000000000001043","DOIUrl":"10.1097/BOR.0000000000001043","url":null,"abstract":"<p><strong>Purpose of review: </strong>Muscle imaging is commonly utilized in idiopathic inflammatory myopathies (IIM) for diagnostic evaluation, selection of muscle biopsy site, and differentiating between disease activity versus damage. In this review, we discuss the current state and recent developments in the use of muscle imaging modalities including muscle magnetic resonance imaging (MRI), ultrasound (US), and positron emission tomography (PET) scan.</p><p><strong>Recent findings: </strong>Muscle MRI is a clinically useful tool in evaluation of IIM with studies showing good correlations between pattern of morphological changes on MRI and histopathological findings on muscle biopsy. The use of computer aided diagnostics to enable quantification of muscle pathology will be a welcome development for future studies and trials. New studies highlight that muscle US could be a particularly useful point of care tool in longitudinal monitoring of patients with active myositis. Muscle FDG-PET scan shows inflammatory activity in IIM muscle and can also provide additional information on extra-muscular manifestations and cancer screening. Utilization of novel tracers is an exciting development for IIM evaluation.</p><p><strong>Summary: </strong>Muscle MRI remains the gold standard for muscle imaging in IIM. Growing literature on muscle US and PET scan highlight their promising applications in IIM.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"445-452"},"PeriodicalIF":5.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142016686","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}