Alana J Haussmann, Zsuzsanna H McMahan, Elizabeth R Volkmann
{"title":"Understanding the gastrointestinal microbiome in systemic sclerosis: methodological advancements and emerging research.","authors":"Alana J Haussmann, Zsuzsanna H McMahan, Elizabeth R Volkmann","doi":"10.1097/BOR.0000000000001048","DOIUrl":"https://doi.org/10.1097/BOR.0000000000001048","url":null,"abstract":"<p><strong>Purpose of review: </strong>This review highlights the role of the gastrointestinal (GI) microbiome in systemic sclerosis (SSc). We describe techniques for evaluating the GI microbiome in humans, and emerging research linking GI microbiome alterations (i.e., dysbiosis) and distinct SSc clinical manifestations. We also address the evolving treatment landscape targeting dysbiosis in SSc.</p><p><strong>Recent findings: </strong>Recent literature brings into focus the complex relationship between the GI microbiome and SSc pathogenesis. Advanced techniques (e.g., shotgun metagenomics, meta-transcriptomics) provide deeper insights into microbial taxonomy and active gene expression, exposing dysbiosis as a potential driver of SSc. New studies demonstrate that SSc patients who possess specific SSc clinical features, (e.g., interstitial lung disease), have unique GI microbiome profiles.</p><p><strong>Summary: </strong>Dysbiosis is associated with specific clinical features in patients with SSc. New tools for studying the GI microbiome have furthered our understanding of the relationship between dysbiosis and SSc complications. Therapeutic avenues such as dietary adjustments, probiotics, antibiotics, mindfulness practices, and fecal transplants offer potential for managing SSc and preventing its progression through GI microbiome modulation. By clarifying what is known about the relationship between the GI dysbiosis, GI dysfunction, and SSc, this review enhances our understanding of SSc pathogenesis and proposes targeted interventions.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2024-08-26","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142072320","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":"Cross-tissue organization of myeloid cells in scleroderma and related fibrotic diseases.","authors":"Ian D Odell","doi":"10.1097/BOR.0000000000001047","DOIUrl":"10.1097/BOR.0000000000001047","url":null,"abstract":"<p><strong>Purpose of review: </strong>Scleroderma and other fibrotic diseases have been investigated using single-cell RNA sequencing (scRNA-Seq), which has demonstrated enrichment in myeloid cell populations in multiple tissues. However, scRNA-Seq studies are inconsistent in their nomenclature of myeloid cell types, including dendritic cells, monocytes, and macrophages. Using cell type-defining gene signatures, I propose a unified nomenclature through analysis of myeloid cell enrichment across fibrotic tissues.</p><p><strong>Recent findings: </strong>scRNA-Seq of human blood and skin identified a new subset of dendritic cells called DC3. DC3 express similar inflammatory genes to monocytes, including FCN1 , IL1B, VCAN, S100A8, S100A9 , and S100A12 . DC3 can be distinguished from monocytes by expression of EREG and Fc receptor genes such as FCER1A and FCGR2B . scRNA-Seq analyses of scleroderma skin and lung, idiopathic pulmonary fibrosis (IPF), COVID-19 lung fibrosis, myelofibrosis, and liver, kidney, and cardiac fibrosis all showed enrichment in myeloid cell types. Although they were called different names, studies of scleroderma skin and lung as well as liver cirrhosis datasets demonstrated enrichment in DC3. By contrast, lung, heart, and kidney fibrosis were enriched in SPP1 macrophages. High numbers of DC3 in the skin was associated with worse SSc skin and lung fibrosis severity.</p><p><strong>Summary: </strong>scRNA-Seq of multiple diseases showed enrichment of DC3 in fibrotic skin, lung, and liver, whereas SPP1 macrophages occurred in fibrotic lung, heart, and kidney. Because DC3 and SPP1 macrophages showed organ-specific enrichment, understanding their signaling mechanisms across tissues will be important for future investigation.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2024-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11451931/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142016685","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":"Current approach to muscle imaging in myositis.","authors":"Didem Saygin, Jemima Albayda","doi":"10.1097/BOR.0000000000001043","DOIUrl":"https://doi.org/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":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2024-08-23","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}
Cristina Ricco, Caroline J Stone, Victoria P Werth
{"title":"Clinical endpoints in myositis: challenges and ways forward.","authors":"Cristina Ricco, Caroline J Stone, Victoria P Werth","doi":"10.1097/BOR.0000000000001044","DOIUrl":"https://doi.org/10.1097/BOR.0000000000001044","url":null,"abstract":"<p><strong>Purpose of review: </strong>This review addresses the challenges and advances in clinical endpoints for myositis, with a particular focus on ensuring comprehensive assessment of both muscle and skin disease activity. The relevance of this review stems from recent developments in outcome measures and their implications for clinical trial design and patient inclusivity. While quality of life (QoL) and lung involvement are also important aspects of myositis, they are beyond the scope of this review and need to be addressed in future studies.</p><p><strong>Recent findings: </strong>Traditional outcome measures like the Total Improvement Score (TIS) have limitations, especially for patients with skin-predominant dermatomyositis (DM). Recent studies highlight the importance of incorporating skin-specific measures such as the Cutaneous Disease Area and Severity Index (CDASI) and the novel composite measure, Dermatomyositis Outcomes for Muscle and Skin (DMOMS). These measures provide a more balanced assessment of disease activity. Clinical trial data analyzed using these measures have demonstrated significant benefits for patients with both classic and amyopathic DM, emphasizing the need for their broader adoption.</p><p><strong>Summary: </strong>Advancements in outcome measures are crucial for inclusive and effective myositis clinical trials. Incorporating comprehensive tools like the DMOMS can enhance the assessment of both muscle and skin disease activities, potentially leading to better therapeutic strategies and improved patient outcomes. This shift is essential for addressing the needs of all Idiopathic inflammatory myopathy patients, including those with skin-predominant DM.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2024-08-23","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142016684","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":"https://doi.org/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":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2024-08-20","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}
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":"https://doi.org/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":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2024-08-14","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}
{"title":"Interstitial lung disease and myositis.","authors":"Takahisa Gono, Masataka Kuwana","doi":"10.1097/BOR.0000000000001037","DOIUrl":"https://doi.org/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":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2024-08-13","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}
Stefanie Glaubitz, Didem Saygin, Ingrid E Lundberg
{"title":"Current efforts and historical perspectives on classification of idiopathic inflammatory myopathies.","authors":"Stefanie Glaubitz, Didem Saygin, Ingrid E Lundberg","doi":"10.1097/BOR.0000000000001042","DOIUrl":"https://doi.org/10.1097/BOR.0000000000001042","url":null,"abstract":"<p><strong>Purpose of review: </strong>The classification of idiopathic inflammatory myopathies is challenging due to the large number of clinical, serological, histopathological and genetic findings, as well as the latest findings and developments in the field of myositis research. The latest official classification criteria are the 2017 European Alliance of Associations for Rheumatology (EULAR)/American College of Rheumatology (ACR) classification criteria for adult and juvenile idiopathic inflammatory myopathies, which have been extensively reviewed in recent years for their applicability, sensitivity and specificity.</p><p><strong>Recent findings: </strong>The sensitivity and specificity of the 2017 ACR/EULAR criteria are sometimes performing better, but usually at the same level as the previous criteria. A large number of further suggestions for amendments to the criteria have been made. In particular there is a need to revise the criteria with regard to the addition of new myositis-specific autoantibodies, newly defined subgroups (especially antisynthetase syndrome, immune medicated necrotizing myopathy and overlap myositis) and possibly the addition of further diagnostic procedures (for instance, muscle MRI or PET CT) to improve the accuracy and timeliness of the criteria.</p><p><strong>Summary: </strong>Efforts to optimize the myositis classification criteria have been extensive in recent years and a new global interdisciplinary collaboration of clinicians is currently taking place based on the previous results with the aim of revising the 2017 EULAR/ACR classification criteria.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2024-08-13","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141916350","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":"Advances in the diagnosis of multiorgan involvement in systemic sclerosis: a focus on MRI.","authors":"Attila Feher, Francesco Del Galdo, Sven Plein","doi":"10.1097/BOR.0000000000001040","DOIUrl":"https://doi.org/10.1097/BOR.0000000000001040","url":null,"abstract":"<p><strong>Purpose of review: </strong>Systemic sclerosis (SSc) is a rare chronic multisystem autoimmune disease characterized by endothelial dysfunction, tissue hypoxia, and diffuse organ fibrosis. MRI provides a radiation free approach to noninvasively assess the key manifestations of SSc in multiple organs. The purpose of this review is to summarize recent advances in MRI techniques to provide diagnostic and prognostic information in patients with SSc.</p><p><strong>Recent findings: </strong>MRI can probe processes that play a key role in the development of SSc-related complications, including neointima proliferation, fibrosis, and hypoxia. Feature tracking and parametric mapping MRI can detect cardiac involvement at the subclinical level. Contrast-free MRI angiography with Digital Artery Volume Index (DAVIX) assessment allow comprehensive assessment of hand involvement. T1 mapping and BOLD imaging can assess SSc effects on skeletal muscle, and lung MRI is becoming a key method for imaging of interstitial lung disease. As a new exciting application, the sodium content of the skin can be quantified by 23Na MRI reflective of glycosaminoglycan content.</p><p><strong>Summary: </strong>Recent advances in MRI provide a unique opportunity to study the key pathophysiologic processes and clinical manifestations of SSc in multiple organs noninvasively, which can pave the way for the development of effective therapies.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2024-08-08","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141901253","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":"Calcinosis in dermatomyositis.","authors":"Srijana Davuluri, Lorinda Chung, Christian Lood","doi":"10.1097/BOR.0000000000001036","DOIUrl":"10.1097/BOR.0000000000001036","url":null,"abstract":"<p><strong>Purpose of review: </strong>To provide the most recent literature on our understanding behind the pathogenesis and the treatment of calcinosis in dermatomyositis.</p><p><strong>Recent findings: </strong>Early diagnosis and controlling the overall disease activity are cornerstones to prevent calcinosis in juvenile dermatomyositis. Observational cohort studies showed that prolonged state of inflammation and features of vascular dysfunction like digital ulcers and abnormal nailfold capillaries are associated with calcinosis. Neutrophil activation and mitochondrial dysfunction have recently emerged as potential mechanistic pathways involved in calcinosis pathogenesis. Few recent case series have alluded to the efficacy of topical and intralesional sodium thiosulfate, while JAK inhibitors appear to be newer promising therapy in juvenile dermatomyositis.</p><p><strong>Summary: </strong>Calcinosis in dermatomyositis consists of deposition of insoluble calcium compounds in the skin and other tissues. It is prevalent in up to 75% of patients with juvenile dermatomyositis and up to 20% in adult dermatomyositis. While it leads to significant patient morbidity, we do not yet understand the pathogenesis in its entirety. Surgical excision although palliative is the mainstay of treatment and should be offered to patients. All available treatment options are only based on very low level of evidence.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":null,"pages":null},"PeriodicalIF":5.2,"publicationDate":"2024-07-25","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11451928/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141906148","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}