Journal of Scleroderma and Related Disorders最新文献

{"title":"Exploratory clinical subgroup clustering in systemic sclerosis Results from the Indian Progressive Systemic Sclerosis Registry","authors":"Shery Susan Philip, R. Janardana, Padmanabha D. Shenoy, C. Kavadichanda, D. Bairwa, G. Sircar, Parasar Ghosh, A. Wakhlu, Sumithra Selvam, Dinesh Khanna, V. Shobha","doi":"10.1177/23971983231215470","DOIUrl":"https://doi.org/10.1177/23971983231215470","url":null,"abstract":"To conduct an exploratory cluster analysis of systemic sclerosis patients from the baseline data of the Indian systemic sclerosis registry. Patients satisfying American College of Rheumatology-European League Against Rheumatism classification criteria for systemic sclerosis were included. The clusters formed using clinical and immunological parameters were compared. Of the 564 systemic sclerosis registry participants, 404 patients were included. We derived four clusters of which three were anti-topoisomerase I predominant and one was anti-centromere antibody 2 dominant. Cluster 1 ( n-82 (20.3%)) had diffuse cutaneous systemic sclerosis patients with the most severe skin disease, anti-topoisomerase I positivity, males, younger age of onset and high prevalence of musculoskeletal, vasculopathic and gastrointestinal features. Cluster 2 ( n-141 (34.9%)) was also diffuse cutaneous systemic sclerosis and anti-topoisomerase I predominant but with less severe skin phenotype than cluster 1 and a lesser prevalence of musculoskeletal, vasculopathic and gastrointestinal features. Cluster 3 ( n-119 (29.5%)) had limited cutaneous systemic sclerosis patients with anti-topoisomerase I positivity along with other antibodies. The proximal muscle weakness was higher and digital pitting scars were lower, while other organ involvement was similar between clusters 2 and 3. Cluster 4 ( n-62 (15.30%)) was the least severe group with limited cutaneous systemic sclerosis and anti-centromere antibody predominance. Age of onset was higher with low musculoskeletal disease and a higher presence of upper gastrointestinal features. The prevalence of interstitial lung disease was similar in the three anti-topoisomerase I predominant clusters. With exploratory cluster analysis, we confirmed the possibility of subclassification of systemic sclerosis along a spectrum based on clinical and immunological characteristics. We also corroborated the presence of anti-topoisomerase I in limited cutaneous systemic sclerosis and the association of interstitial lung disease with anti-topoisomerase I.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-12-14","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"139003253","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Paul Klee’s progressive dyspnea: A series of historical and clinical vignettes, part V","authors":"Richard M Silver","doi":"10.1177/23971983231213140","DOIUrl":"https://doi.org/10.1177/23971983231213140","url":null,"abstract":"Paul Klee (1879–1940), the 20th-century Swiss-German artist, suffered and died from complications of systemic sclerosis (SSc, scleroderma). This is the fifth in a series of clinical and historical vignettes wherein Klee’s cardiopulmonary symptoms are described with an emphasis on how progressive dyspnea impacted Klee’s life.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"138597966","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Use of exercise tests in screening for pulmonary arterial hypertension in systemic sclerosis: A systematic literature review","authors":"Sarah Madigan, Susanna Proudman, David Schembri, Huw Davies, Robert Adams","doi":"10.1177/23971983231199148","DOIUrl":"https://doi.org/10.1177/23971983231199148","url":null,"abstract":"Background and objective: Patients with systemic sclerosis (SSc) and pulmonary arterial hypertension (PAH) have a poor prognosis, accounting for 30% of all SSc-related deaths. Guidelines recommend annual screening for PAH regardless of symptoms, as early treatment improves outcomes. Current protocols include combinations of clinical features, biomarkers, pulmonary function tests, and echocardiography. None include exercise testing, although early-stage PAH may only be evident during exercise. This systematic review assessed the performance of exercise tests in predicting the presence of PAH in patients with SSc, where PAH was confirmed through right heart catheterisation (RHC). Methods: Comprehensive literature searches were performed using MEDLINE, EMBASE, Cochrane Database of Systematic Reviews and Cochrane Central Register of Controlled Trails, CINAHL, Scopus and Web of Science from inception to May 2023. Articles were screened for eligibility by two independent reviewers. Eligibility criteria included the use of a non-invasive exercise test to screen adult patients to detect PAH in a population without a previous diagnosis of PAH, with diagnosis confirmed by RHC. Results: Eight studies met the inclusion criteria, describing at least one of three different non-invasive exercise tests: cardiopulmonary exercise test, six-minute walk test and stress Doppler echocardiography. All studies found that exercise tests had some ability to predict the presence of PAH, with sensitivity between 50% and 100% and specificity from 73% to 91%. Conclusion: Exercise tests are infrequently used for screening for PAH in SSc but can predict the presence of PAH. More data are required to establish which tests are most effective.","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":0.0,"publicationDate":"2023-10-02","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"135899953","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Nailfold capillaroscopy and candidate-biomarker levels in systemic sclerosis-associated pulmonary hypertension: A cross-sectional study.","authors":"Jacqueline Mj Lemmers,&nbsp;Arjan Pm van Caam,&nbsp;Brigit Kersten,&nbsp;Cornelia Hm van den Ende,&nbsp;Hanneke Knaapen,&nbsp;Arie Pj van Dijk,&nbsp;Wanda Hagmolen Of Ten Have,&nbsp;Frank Hj van den Hoogen,&nbsp;Hans Koenen,&nbsp;Sander I van Leuven,&nbsp;Wynand Alkema,&nbsp;Ruben L Smeets,&nbsp;Madelon C Vonk","doi":"10.1177/23971983231175213","DOIUrl":"https://doi.org/10.1177/23971983231175213","url":null,"abstract":"<p><strong>Objectives: </strong>Pulmonary hypertension is one of the leading causes of death in systemic sclerosis. Early detection and treatment of pulmonary hypertension in systemic sclerosis is crucial. Nailfold capillaroscopy microscopy, vascular autoantibodies AT1R and ETAR, and several candidate-biomarkers have the potential to serve as noninvasive tools to identify systemic sclerosis patients at risk for developing pulmonary hypertension. Here, we explore the classifying potential of nailfold capillaroscopy microscopy characteristics and serum levels of selected candidate-biomarkers in a sample of systemic sclerosis patients with and without different forms of pulmonary hypertension.</p><p><strong>Methods: </strong>A total of 81 consecutive systemic sclerosis patients were included, 40 with systemic sclerosis pulmonary hypertension and 41 with no pulmonary hypertension. In each group, quantitative and qualitative nailfold capillaroscopy microscopy characteristics, vascular autoantibodies AT1R and ETAR, and serum levels of 24 soluble serum factors were determined. For evaluation of the nailfold capillaroscopy microscopy characteristics, linear regression analysis accounting for age, sex, and diffusing capacity of the lungs for carbon monoxide percentage predicted was used. Autoantibodies and soluble serum factor levels were compared using two-sample <i>t</i> test with equal variances.</p><p><strong>Results: </strong>No statistically significant differences were observed in quantitative or qualitative nailfold capillaroscopy microscopy characteristics, or vascular autoantibody ETAR and AT1R titer between systemic sclerosis-pulmonary hypertension and systemic sclerosis-no pulmonary hypertension. In contrast, several serum levels of soluble factors differed between groups: Endostatin, sVCAM, and VEGFD were increased, and CXCL4, sVEGFR2, and PDGF-AB/BB were decreased in systemic sclerosis-pulmonary hypertension. Random forest classification identified Endostatin and CXCL4 as the most predictive classifiers to distinguish systemic sclerosispulmonary hypertension from systemic sclerosis-no pulmonary hypertension.</p><p><strong>Conclusion: </strong>This study shows the potential for several soluble serum factors to distinguish systemic sclerosis-pulmonary hypertension from systemic sclerosis-no pulmonary hypertension. We found no classifying potential for qualitative or quantitative nailfold capillaroscopy microscopy characteristics, or vascular autoantibodies.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10515989/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41124450","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Anti-thymocyte globulin exposure in patients with diffuse cutaneous systemic sclerosis undergoing autologous haematopoietic stem cell transplantation.","authors":"Yu-Hsiang Chiu,&nbsp;Anouk Drijver,&nbsp;Rick Admiraal,&nbsp;Anna van Rhenen,&nbsp;Stefan Nierkens,&nbsp;Jacob M van Laar,&nbsp;Julia Spierings","doi":"10.1177/23971983231188232","DOIUrl":"https://doi.org/10.1177/23971983231188232","url":null,"abstract":"<p><strong>Introduction: </strong>Autologous haematopoietic stem cell transplantation improves event-free survival and lung function and reduces skin thickening in patients with progressive diffuse cutaneous systemic sclerosis. Anti-thymocyte globulin is a key lymphoablative constituent of conditioning protocols and is administered in a weight-based dosage. However, whether anti-thymocyte globulin exposure contributes to response to autologous haematopoietic stem cell transplantation and lymphocyte reconstitution in diffuse cutaneous systemic sclerosis patients is unknown. We aimed to explore the relationship between anti-thymocyte globulin exposure, lymphocyte reconstitution and treatment response in diffuse cutaneous systemic sclerosis patients undergoing autologous haematopoietic stem cell transplantation.</p><p><strong>Methods: </strong>A retrospective cohort of 15 diffuse cutaneous systemic sclerosis patients undergoing autologous haematopoietic stem cell transplantation was performed. Clinical characteristics and routine laboratory results were retrieved from electronic medical records. Anti-thymocyte globulin concentrations were measured in cryopreserved plasma samples at four time points (day 1 and week 1, 2 and 4) after stem cell reinfusion. Anti-thymocyte globulin exposure was estimated using a validated population pharmacokinetic model.</p><p><strong>Results: </strong>During a median follow-up of 45 months (interquartile range 19-66), 11 (73%) patients had a treatment response, and 4 (27%) were non-responders. Although all patients received the same weight-based anti-thymocyte globulin dosage, 7.5 mg/kg divided over 3 days, anti-thymocyte globulin exposure varied. Anti-thymocyte globulin exposure was higher in responders than in non-responders (163 AU*day/mL (interquartile range 153-183) and 137 AU*day/mL (interquartile range 101-149), respectively, <i>p</i> = .026). Anti-thymocyte globulin exposure was not correlated with lymphocyte reconstitution or infection rate.</p><p><strong>Conclusion: </strong>Weight-based dosing of anti-thymocyte globulin results in variable anti-thymocyte globulin exposure and treatment response across individuals.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10515999/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41134026","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Support and information needs of people with systemic sclerosis by time since diagnosis: A cross-sectional study.","authors":"Sabrina Provencher,&nbsp;Richard S Henry,&nbsp;Carolina Bacalao,&nbsp;Marie-Eve Carrier,&nbsp;Linda Kwakkenbos,&nbsp;Brett D Thombs","doi":"10.1177/23971983231181726","DOIUrl":"https://doi.org/10.1177/23971983231181726","url":null,"abstract":"<p><strong>Background: </strong>How support and informational needs of people with systemic sclerosis (SSc) may differ by time since diagnosis is not known. Our objective was to determine if informational and support needs of recently diagnosed individuals with systemic sclerosis differ from people diagnosed for longer periods of time.</p><p><strong>Methods: </strong>The North American Scleroderma Support Group Members survey included 30 items on reasons for attending support groups. Respondents were classified by time since diagnosis of 0-3 years, 4-9 years or 10+ years. Survey item responses were dichotomized into <i>Not Important</i> or <i>Somewhat Important</i> versus <i>Important</i> or <i>Very Important</i>. We conducted Chi-square tests with Hochberg's Sequential Method to identify item differences by time since diagnosis.</p><p><strong>Results: </strong>A total of 175 respondents completed the survey. Most support needs were rated as <i>Important</i> or <i>Very Important</i> by respondents, regardless of disease duration, particularly needs related to interpersonal and social support (10 items; median 81%) and learning about disease treatment and management strategies (11 items; median 82%). Discussing other aspects of living with systemic sclerosis (e.g. spirituality, discussing disease with family and friends) was rated lower (9 items; 44%). Respondents with 0-3 years since diagnosis were the highest on 29 of 30 items. Respondents with 0-3 years since diagnosis were significantly higher on items related to discussing medical care and 4 items on other aspects (spirituality, talking with family and friends, financial issues, sexual issues).</p><p><strong>Conclusion: </strong>People with systemic sclerosis have a wide range of information and support needs, regardless of their disease duration, but people with recent diagnoses have greater needs.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":2.0,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://ftp.ncbi.nlm.nih.gov/pub/pmc/oa_pdf/8d/2a/10.1177_23971983231181726.PMC10515994.pdf","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41162890","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Scleroderma renal crisis triggered by ibuprofen: Insights on complement-directed therapy.","authors":"Prochore Kamgang Semeu, Maxime Taghavi, Caroline Geers, Luc Mouthon, Leonel Barreto Gutierrez, Patrick Stordeur","doi":"10.1177/23971983231163663","DOIUrl":"10.1177/23971983231163663","url":null,"abstract":"<p><p>Scleroderma renal crisis is a severe complication of systemic sclerosis with a poor prognosis. Therefore, identifying precipitating factors is essential. Among known risk factors, only few are reversible. On the contrary, anti-C5 therapy appears effective, at least in some cases. We describe a 59-year-old man with diffuse cutaneous systemic sclerosis who developed life-threatening scleroderma renal crisis following ibuprofen administration. Despite aggressive management, he did not improve. Renal biopsy have displayed features of thrombotic microangiopathy but no complement deposition. We then discuss the pathomechanism of scleroderma renal crisis that could drive eculizumab treatment since some renal biopsies exhibit complement deposits and others do not.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10515997/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41130919","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Therapeutic effects of thalidomide on patients with systemic sclerosis-associated interstitial lung disease.","authors":"Jie Pan, Fei Dong, Li Ma, Cheng Zhao, Fang Qin, Jing Wen, Wanling Wei, Ling Lei","doi":"10.1177/23971983231180077","DOIUrl":"10.1177/23971983231180077","url":null,"abstract":"<p><strong>Objective: </strong>To evaluate the clinical efficacy of thalidomide in patients with systemic sclerosis-associated interstitial lung disease.</p><p><strong>Methods: </strong>Ninety-six systemic sclerosis-associated interstitial lung disease patients who received basic glucocorticoid treatment and admitted between 2016 and 2020 were included in this study, including 48 cases in the thalidomide group (combination of thalidomide and cyclophosphamide) and 48 cases in control group (cyclophosphamide monotherapy). Evaluation items included clinical symptoms, modified Rodnan skin score, pulmonary function test, chest high-resolution computed tomography scores, and adverse effects between two groups after 24 weeks of treatment.</p><p><strong>Results: </strong>Remarkable improvements in several aspects were found in the thalidomide group, including modified Rodnan skin score, expiratory dyspnea score, cough visual analog scale score, total ground-glass opacity score, and total interstitial lung disease score. Compared to the control group, improvements in the thalidomide group were found, such as significantly decreased cough visual analog scale score and expectoration; increased number of platelets; improved pulmonary fibrosis (<i>p</i> <i>=</i> 0.056), and reduced carbon monoxide diffusing capacity (<i>p</i> = 0.053). There were no statistically significant differences in the expiratory dyspnea score and predicted forced vital capacity between the two groups. Patients who experienced at least one adverse event in the control group and thalidomide group were 33.3% and 64.6% (<i>p</i> = 0.002); while those with serious adverse events were 8.3% versus 12.5% (<i>p</i> = 0.504). Venous thrombosis was found in one case in the thalidomide group.</p><p><strong>Conclusion: </strong>Thalidomide combined with cyclophosphamide can improve the symptoms of cough and expectoration in patients with systemic sclerosis-associated interstitial lung disease, and may slightly delay the progression of pulmonary fibrosis, but with the possibility of an increased risk of adverse events.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10515992/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41139654","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Application and performance of disease activity indices proposed for patients with systemic sclerosis in an international cohort of patients with juvenile systemic sclerosis.","authors":"Jens Klotsche, Kathryn S Torok, Ozgur Kasapcopur, Amra Adrovic, Maria Teresa Terreri, Ana Paula Sakamoto, Maria Katsicas, Flavio Sztajnbok, Edoardo Marrani, Alberto Sifuentes-Giraldo, Valda Stanevicha, Jordi Anton, Brian Feldmann, Mikhail Kostik, Dana Nemcova, Maria Jose Santos, Simone Appenzeller, Tadej Avcin, Cristina Battagliotti, Lillemor Berntson, Blanca Bica, Jürgen Brunner, Despina Eleftheriou, Liora Harel, Gerd Horneff, Tilmann Kallinich, Kirsten Minden, Susan Nielsen, Anjali Patwardhan, Nicola Helmus, Ivan Foeldvari","doi":"10.1177/23971983231164700","DOIUrl":"10.1177/23971983231164700","url":null,"abstract":"<p><strong>Objectives: </strong>Juvenile systemic sclerosis is a rare childhood disease. Three disease activity indices have been published for adult patients with systemic sclerosis: the European Scleroderma Study Group Index, a modified version of the European Scleroderma Study Group Index and the revised European Scleroderma Trials and Research index. The objective of this study was to determine the feasibility and performance of the three disease activity indices in a prospectively followed cohort of patients with juvenile systemic sclerosis.</p><p><strong>Methods: </strong>The analysis cohort was selected from the prospective international inception cohort enrolling juvenile systemic sclerosis patients. The correlation of the disease activity indices with the physicians' and the patients' global assessment of disease activity was determined. The disease activity indices were compared between patients with active and inactive disease. Sensitivity to change between 6- and 12-month follow-up was investigated by mixed models.</p><p><strong>Results: </strong>Eighty percent of the 70 patients had a diffuse cutaneous subtype. The revised European Scleroderma Trials and Research index was highly correlated with the physician-reported global disease activity/parents-reported global disease activity (r = 0.74/0.64), followed by the European Scleroderma Study Group activity index (r = 0.61/0.55) and the modified version of the European Scleroderma Study Group activity index (r = 0.51/0.43). The disease activity indices significantly differed between active and inactive patients. The disease activity indices showed sensitivity to change between 6- and 12-month follow-up among patients who improved or worsened according to the physician-reported global disease activity and the parents-reported global disease activity.</p><p><strong>Conclusion: </strong>Overall, no disease activity score is superior to the other, and all three scores have limitations in the application in juvenile systemic sclerosis patients. Furthermore, research on the concept of disease activity and suitable scores to measure disease activity in patients with juvenile systemic sclerosis is necessary in future.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10515993/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41134027","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Consensus on the assessment of systemic sclerosis-associated primary heart involvement: World Scleroderma Foundation/Heart Failure Association guidance on screening, diagnosis, and follow-up assessment.","authors":"Cosimo Bruni, Maya H Buch, Aleksandra Djokovic, Giacomo De Luca, Raluca B Dumitru, Alessandro Giollo, Ilaria Galetti, Alexia Steelandt, Konstantinos Bratis, Yossra Atef Suliman, Ivan Milinkovic, Anna Baritussio, Ghadeer Hasan, Anastasia Xintarakou, Yohei Isomura, George Markousis-Mavrogenis, Sophie Mavrogeni, Luna Gargani, Alida Lp Caforio, Carsten Tschöpe, Arsen Ristic, Sven Plein, Elijah Behr, Yannick Allanore, Masataka Kuwana, Christopher P Denton, Daniel E Furst, Dinesh Khanna, Thomas Krieg, Renzo Marcolongo, Alessia Pepe, Oliver Distler, Petros Sfikakis, Petar Seferovic, Marco Matucci-Cerinic","doi":"10.1177/23971983231163413","DOIUrl":"10.1177/23971983231163413","url":null,"abstract":"<p><strong>Introduction: </strong>Heart involvement is a common problem in systemic sclerosis. Recently, a definition of systemic sclerosis primary heart involvement had been proposed. Our aim was to establish consensus guidance on the screening, diagnosis and follow-up of systemic sclerosis primary heart involvement patients.</p><p><strong>Methods: </strong>A systematic literature review was performed to investigate the tests used to evaluate heart involvement in systemic sclerosis. The extracted data were categorized into relevant domains (conventional radiology, electrocardiography, echocardiography, cardiac magnetic resonance imaging, laboratory, and others) and presented to experts and one patient research partner, who discussed the data and added their opinion. This led to the formulation of overarching principles and guidance statements, then reviewed and voted on for agreement. Consensus was attained when the mean agreement was ⩾7/10 and of ⩾70% of voters.</p><p><strong>Results: </strong>Among 2650 publications, 168 met eligibility criteria; the data extracted were discussed over three meetings. Seven overarching principles and 10 guidance points were created, revised and voted on. The consensus highlighted the importance of patient counseling, differential diagnosis and multidisciplinary team management, as well as defining screening and diagnostic approaches. The initial core evaluation should integrate history, physical examination, rest electrocardiography, trans-thoracic echocardiography and standard serum cardiac biomarkers. Further investigations should be individually tailored and decided through a multidisciplinary management. The overall mean agreement was 9.1/10, with mean 93% of experts voting above 7/10.</p><p><strong>Conclusion: </strong>This consensus-based guidance on screening, diagnosis and follow-up of systemic sclerosis primary heart involvement provides a foundation for standard of care and future feasibility studies that are ongoing to support its application in clinical practice.</p>","PeriodicalId":17036,"journal":{"name":"Journal of Scleroderma and Related Disorders","volume":null,"pages":null},"PeriodicalIF":1.4,"publicationDate":"2023-10-01","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10515996/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"41141896","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":0,"RegionCategory":"","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}