{"title":"Autologous hematopoietic stem cell transplant for systemic sclerosis associated interstitial lung disease.","authors":"Jana Zielonka, Jean Paul Higuero Sevilla","doi":"10.1097/BOR.0000000000001050","DOIUrl":"10.1097/BOR.0000000000001050","url":null,"abstract":"<p><strong>Purpose of review: </strong>Over the last 25 years, the role of autologous hematopoietic stem cell transplant (HSCT) in the treatment of diffuse cutaneous systemic sclerosis (dcSSc) has been elucidated. However, multiple critical questions remain regarding this therapy. Of particular interest is the role of HSCT in the treatment of systemic sclerosis (SSc)-associated interstitial lung disease since this is the leading cause of death in SSc.</p><p><strong>Recent findings: </strong>Most clinical trials and observational studies of HSCT for the treatment of dcSSc have reported pulmonary outcomes as secondary outcomes, Also, most studies have excluded patients with significant pulmonary function impairment. Despite these limitations, there is increasing evidence that suggests that HSCT leads to interstitial lung disease stabilization and possibly improvement of lung function based on pulmonary function tests and imaging.</p><p><strong>Summary: </strong>HSCT has demonstrated improved long-term outcomes compared to conventional therapies for dcSSC. Future research is needed to refine or expand patient selection, optimize conditioning regimens, and evaluate the potential role of maintenance immunosuppression. We recommend an increased focus on interstitial lung disease since this is the primary cause of death in SSc.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"410-419"},"PeriodicalIF":5.2,"publicationDate":"2024-11-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142343413","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":"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 23 Na 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":" ","pages":"387-392"},"PeriodicalIF":5.2,"publicationDate":"2024-11-01","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":" ","pages":"453-458"},"PeriodicalIF":5.2,"publicationDate":"2024-11-01","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}
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":"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":" ","pages":"430-437"},"PeriodicalIF":5.2,"publicationDate":"2024-11-01","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}
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":"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":" ","pages":"473-480"},"PeriodicalIF":5.2,"publicationDate":"2024-11-01","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":"Juvenile Behçet syndrome: a contemporary view and differential diagnosis in pediatric practice.","authors":"Mehmet Yildiz,Oya Koker,Ozgur Kasapcopur","doi":"10.1097/bor.0000000000001057","DOIUrl":"https://doi.org/10.1097/bor.0000000000001057","url":null,"abstract":"PURPOSE OF REVIEWThis review aims to provide a comprehensive and contemporary overview of juvenile Behçet syndrome (jBS), highlighting its clinical manifestations, diagnostic challenges, and treatment strategies.RECENT FINDINGSBehçet syndrome, with its intricate etiopathogenesis and diverse clinical phenotypes, is more aptly classified as a syndrome than a single disease. Its heterogeneous nature requires a broad diagnostic approach and sophisticated differential diagnosis capabilities. The relatively rare occurrence of Behçet syndrome, combined with incomplete clinical presentations and overlapping differential diagnoses, presents significant diagnostic challenges, particularly in pediatric cases. Nevertheless, substantial progress has been made in treatment, especially in managing inflammatory components and preventing complications. Juvenile patients, given their developmental stage, require distinct therapeutic strategies compared to adults, with careful consideration of treatment side effects on growth and psychosocial development.SUMMARYTo ensure early identification of jBS, it is imperative to refine and develop diagnostic criteria specifically tailored to pediatric populations. With a deeper understanding of the disease mechanisms, treatment protocols should be designed to address the developmental, psychosocial, and individual needs of patients, aiming to minimize long-term side effects. Additionally, comprehensive studies considering age, sex, and ethnic differences are necessary to fill gaps in the literature and resolve existing inconsistencies.","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":"17 1","pages":""},"PeriodicalIF":5.1,"publicationDate":"2024-09-19","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142250187","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":2,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}
{"title":"The role of neutrophil extracellular traps in inflammatory rheumatic diseases.","authors":"Norio Hanata,Mariana J Kaplan","doi":"10.1097/bor.0000000000001054","DOIUrl":"https://doi.org/10.1097/bor.0000000000001054","url":null,"abstract":"PURPOSE OF REVIEWDysregulation in neutrophil extracellular trap (NET) formation and degradation has been reported in several inflammatory rheumatic diseases. This review summarizes the recent advances in the understanding the role of NETs in the context of inflammatory rheumatic diseases.RECENT FINDINGSNET formation is enhanced in peripheral blood of patients with large vessel vasculitis and polymyalgia rheumatica. NETs are detected in affected organs in autoimmune conditions, and they might play pathological roles in tissues. Several understudied medications and supplements suppress NET formation and ameliorate animal models of inflammatory rheumatic diseases. NETs and anti-NET antibodies have potential utility as disease biomarkers.SUMMARYGrowing evidence has suggested the contribution of NET dysregulation to the pathogenesis of several inflammatory rheumatic diseases. Further research is warranted in regard to clinical impact of modulating aberrant NET formation and clearance in inflammatory rheumatic diseases.","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":"153 1","pages":""},"PeriodicalIF":5.1,"publicationDate":"2024-09-12","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142194172","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":"Renal disease in pediatric rheumatology.","authors":"Ellen Cody, Hermine I Brunner","doi":"10.1097/BOR.0000000000001027","DOIUrl":"10.1097/BOR.0000000000001027","url":null,"abstract":"<p><strong>Purpose of review: </strong>This review will provide updates in the outcomes in the common rheumatologic diseases with kidney involvement. Covered are also advances in therapeutics for the use of pediatric rheumatologic diseases with kidney involvement, as well as the potential kidney complications from other rheumatologic diseases and their medications.</p><p><strong>Recent findings: </strong>Two of the more common rheumatologic diseases with kidney involvement, lupus and vasculitis, continue to show inadequate response to initial therapy of renal disease and practice continues to be driven by results of adult studies.</p><p><strong>Summary: </strong>There is a continued need for pediatric specific studies in rheumatologic diseases with kidney involvement as outcomes continue to be inadequate. Despite recently approved treatments for adults with rheumatic diseases and kidney involvement, therapeutic options in pediatrics remain limited, contributing to the overall morbidity and mortality.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"322-327"},"PeriodicalIF":5.2,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"140944497","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}
Elena Galli, Francesco Muratore, Kenneth J Warrington
{"title":"Current management of giant cell arteritis and its complications.","authors":"Elena Galli, Francesco Muratore, Kenneth J Warrington","doi":"10.1097/BOR.0000000000001029","DOIUrl":"10.1097/BOR.0000000000001029","url":null,"abstract":"<p><strong>Purpose of review: </strong>This review provides an update on current management strategies for giant cell arteritis (GCA), emphasizing the need for alternative therapies to reduce disease relapses and mitigate glucocorticoid (GC)-related morbidity.</p><p><strong>Recent findings: </strong>The standard of care for GCA has traditionally involved prolonged use of GC, and recent studies are exploring faster GC tapering regimens in an effort to reduce adverse effects while maintaining disease control. Randomized clinical trials have highlighted the efficacy of tocilizumab (TCZ), an interleukin-6 receptor inhibitor, in reducing disease flares and sparing GCs. However, the optimal treatment duration with TCZ is unknown and patients remain at risk of relapse after treatment discontinuation. An unmet therapeutic need persists for patients who are not candidates for TCZ, and for those who have inadequate response to this biologic. Therefore, investigations into alternative therapies such as targeting interleukin-17A, blocking T-cell activation or inhibiting the Janus kinase-signal transducer and activator of transcription pathway, showcase potential avenues for tailored treatments.</p><p><strong>Summary: </strong>While GCs remain the cornerstone of therapy, TCZ emerges as a promising GC-sparing agent. Ongoing research targeting different pathways implicated in GCA pathogenesis have led to encouraging results. However, the preliminary nature of these findings necessitates larger randomized controlled trials to establish their efficacy conclusively.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"344-350"},"PeriodicalIF":5.2,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141450040","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":"SGLT-2 inhibitors: new horizons for rheumatologists.","authors":"Katherine Chakrabarti, W Joseph McCune","doi":"10.1097/BOR.0000000000001030","DOIUrl":"10.1097/BOR.0000000000001030","url":null,"abstract":"<p><strong>Purpose of review: </strong>Sodium glucose cotransporter 2 (SGLT2) inhibitors are a class of medications initially developed for the treatment of diabetes, although their cardiac and renal protective benefits are far reaching. There has been marked interest in the rheumatology community to adopt these medications into our clinical practice, particularly for chronic kidney disease with persistent proteinuria.</p><p><strong>Recent findings: </strong>SGLT2 inhibitors have been approved for patients with type 2 diabetes mellitus, heart failure with reduced or preserved ejection fraction, atherosclerotic cardiovascular disease in the setting of type 2 diabetes mellitus, as well as chronic kidney disease with proteinuria. Large studies on SGLT2 inhibitors have largely excluded patients with proteinuric chronic kidney disease due to autoimmune glomerulonephritis due to concerns for confounding from immunosuppression. The Dapagliflozin and Prevention of Adverse Outcomes in CKD Trial (DAPA-CKD) showed that SGLT2 inhibition decreased progression of renal disease in patients with IgA nephropathy. Expanding this to other autoimmune glomerulonephropathies, several small studies have shown improvements in proteinuria in patients with lupus nephritis treated with SGLT2 inhibitors. A study evaluating safety of SGLT2 inhibitors in patients with lupus identified no specific concerns even with concomitant use of immunosuppression.</p><p><strong>Summary: </strong>Small studies have shown that SGLT2 inhibitors can been utilized safely and efficaciously in patients with lupus nephritis. Additional research is needed to identify where these medications fit into the rheumatology treatment armamentarium.</p>","PeriodicalId":11145,"journal":{"name":"Current opinion in rheumatology","volume":" ","pages":"351-359"},"PeriodicalIF":5.2,"publicationDate":"2024-09-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11296270/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"141616079","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}