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A retrospective study enrolled adults (≥18 years) diagnosed with myositis interstitial lung disease (ILD) at a university clinic from 2019–2022. PH was defined using transthoracic echocardiography with tricuspid regurgitation velocity (TRV) ≥2.9 m/s and intermediate probability per 2022 ESC/ERS guidance. Intermediate-to-high probability patients showed higher dermato-specific antibody prevalence, lower FVC and DLCO, and greater mortality and lung-transplantation rates. Findings suggest increased PH risk with reduced lung function and worse outcomes in MSA/MAA-positive patients.",{"@graph":69,"@context":122},[70,84,105],{"@type":71,"itemListElement":72},"BreadcrumbList",[73,77,79,82],{"item":74,"name":75,"@type":76,"position":8},"https://docshare.wps.com","Home","ListItem",{"item":78,"name":9,"@type":76,"position":14},"https://docshare.wps.com/document/",{"item":80,"name":35,"@type":76,"position":81},"https://docshare.wps.com/document/healthcare/",3,{"item":83,"name":65,"@type":76,"position":19},"https://docshare.wps.com/document/echocardiographic-pulmonary-hypertension-in-patients-positive-for-myositis-specific-and-myositis-associated-antibodies/461510/",{"url":83,"name":65,"@type":85,"image":86,"author":91,"headline":65,"publisher":94,"fileFormat":97,"inLanguage":63,"description":67,"dateModified":98,"datePublished":99,"encodingFormat":97,"isAccessibleForFree":100,"interactionStatistic":101},"DigitalDocument",{"url":87,"@type":88,"width":89,"height":90},"https://docshare.wps.com/thumbnails/echocardiographic-pulmonary-hypertension-in-patients-positive-for-myositis-specific-and-myositis-associated-antibodies/461510.png","ImageObject",300,407,{"name":92,"@type":93},"SANS","Person",{"url":74,"name":95,"@type":96},"DocShare","Organization","application/pdf","2026-10-08","2026-09-30",true,{"@type":102,"interactionType":103,"userInteractionCount":39},"InteractionCounter",{"@type":104},"ViewAction",{"@type":106,"mainEntity":107},"FAQPage",[108,114,118],{"name":109,"@type":110,"acceptedAnswer":111},"How was pulmonary hypertension (PH) assessed in the study?","Question",{"text":112,"@type":113},"PH was defined by transthoracic echocardiography using tricuspid regurgitation velocity (TRV) ≥2.9 m/s, consistent with intermediate probability per 2022 ESC/ERS guidelines.","Answer",{"name":115,"@type":110,"acceptedAnswer":116},"What differences were observed between low-probability and intermediate-to-high probability PH groups?",{"text":117,"@type":113},"The intermediate-to-high probability group had higher dermato-specific antibody prevalence, lower FVC and DLCO, and higher all-cause mortality and lung transplantation rates.",{"name":119,"@type":110,"acceptedAnswer":120},"Did immunosuppression status change mortality outcomes in the intermediate-to-high probability group?",{"text":121,"@type":113},"No significant difference in all-cause mortality was found between patients on immunosuppression versus those not on immunosuppression within the intermediate-to-high probability PH group.","https://schema.org",{"og:url":83,"og:type":124,"og:title":65,"og:site_name":95,"og:description":67},"article",{"robots":126,"canonical":83},"index,follow",{"doc_id":128,"site_id":62},461510,1791018287,{"code":4,"msg":5,"data":131},{"doc_id":128,"user_id":132,"nickname":92,"user_avatar":133,"doc_module":4,"category_id":34,"category_name":35,"doc_title":65,"doc_description":67,"doc_content":134,"file_id":135,"file_url":136,"file_type":137,"file_size":138,"view_count":39,"is_deleted":4,"is_public":8,"is_downloadable":8,"audit_status":8,"page_count":139,"language":140,"language_code":63,"site_id":62,"html_lang":63,"table_of_contents":141,"faqs":142,"seo_title":143,"seo_description":67,"update_tm":144,"read_time":145},962090760505,"https://ap-avatar.wpscdn.com/davatar_155a257f0dc6eb9ab79c44ca47cae57d","Article  \nEchocardiographic Pulmonary Hypertension in Patients Positive for Myositis-Specific and Myositis-Associated Antibodies  \nKristina Akopyan 1, *, Jessica Peterson 2, Oluyemisi Amoda 3, Majd Khasawneh 3, Susheela Hadigal 3, Christopher Harden 3, Diana Gomez Manjarres 3, Raju Reddy 4 and Faye Pais 3  \nAcademic Editors: Gabor G. Kovacs and Anna Birnhuber  \nReceived: 21 November 2025  \nRevised: 16 December 2025  \nAccepted: 17 December 2025  \nPublished: 22 December 2025  \nCopyright: © 2025 by the authors. Licensee MDPI, Basel, Switzerland. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution (CC BY) license.  \n1 Division of Hospital Medicine, Department of Medicine, University of Florida, Gainesville, FL 32610-0238, USA  \n2 Malcom Randall Department of Veterans Affairs Medical Center, Gainesville, FL 32610-0238, USA  \n3 Department of Medicine, Division of Pulmonary, Critical Care and Sleep Medicine, University of Florida, Gainesville, FL 32610-0238, USA  \n4 Department of Medicine, Division of Pulmonary and Critical Care Medicine, University of Texas at Austin, Austin, TX 78712, USA  \n* [Correspondence: akopyank@ufl.edu](Correspondence: akopyank@ufl.edu)  \nAbstract  \nBackground: The prevalence of pulmonary hypertension (PH) in patients who are positive for myositis-specific antibody (MSA) and myositis-associated antibody (MAA) remains unclear. Methods: We conducted a retrospective study of patients with an age of 18 years or older diagnosed with myositis interstitial lung disease (ILD) at our university’s ILD clinic between 2019 and 2022 . Echocardiographic PH was defined by tricuspid regurgitation velocity (TRV) ≥ 2.9 m/s on transthoracic echocardiography (TTE) consistent with intermediate probability of PH using 2022 European Society of Cardiology/European Respiratory Society (ESC/ERS) guidelines. We grouped patients based on low probability of PH vs. intermediate to high probability of PH. We examined 6 min walk test (6MWT) data, pulmonary function tests (PFTs), all-cause mortality, and rate of lung transplantation. We also evaluated patients who were on immunosuppression vs. those not on immunosuppression. Results: The intermediate to high probability of PH group had a higher prevalence of dermato-specific antibodies (14.2% vs. 34.5%, p = 0.048) . Specifically, MDA-5 was found to be more prevalent in patients with intermediate to high probability of PH (7.1% vs. 24.1%, p = 0.040) . There was no difference in 6MWT parameters between groups (363.2 ± 115.6 m vs. 294.9 ± 147.5 m, p = 0.108) . FVC and DLCO were lower in patients with intermediate to high probability of PH (71.3 ± 22.4 L vs. 58.8 ± 16.7 L, p = 0.037; 56.3 ± 21.8 mL/min/mmHg vs. 36.9 ± 15.5 mL/min/mmHg, p = 0.003) . The all-cause mortality and rate of lung transplantation was higher in the intermediate to high probability of PH group (5.4% vs. 20.7%, p = 0.041, 0% vs. 6.9%, p = 0.049) . There was no difference in all-cause mortality between patients who were on immunosuppression vs. those who were not on immunosuppression in patients with intermediate to high probability of PH (33.3% vs. 7.1%; p = 0.169) . Conclusions: Patients with MSA/MAA may have an increased risk of PH with reduced lung function, higher mortality, and greater rate of lung transplantation.  \nOur study further elucidates the growing body of evidence that dermato-specific antibodies, such as MDA-5 are associated with an increased risk of PH. Further research is needed to investigate the role of PH and immunosuppression in these patients.  \nKeywords: pulmonary hypertension; myositis-specific antibody; myositis-associated antibody; interstitial lung disease  \n1. Introduction  \nInterstitial lung disease (ILD) has a high prevalence in patients with myositis-specific antibody (MSA) and myositis-associated antibody (MAA). Pulmonary hypertension (PH) is a condition that leads to increased morbidity and mortality in patients with ILD. PH","cbCaisxB2f5dAVZF","https://ap.wps.com/l/cbCaisxB2f5dAVZF","pdf",578051,13,"English","# Abstract\n# Background\n# Methods\n## Study population and definitions\n## Outcomes assessed\n# Results\n# Conclusions","[{\"question\":\"How was pulmonary hypertension (PH) assessed in the study?\",\"answer\":\"PH was defined by transthoracic echocardiography using tricuspid regurgitation velocity (TRV) ≥2.9 m/s, consistent with intermediate probability per 2022 ESC/ERS guidelines.\"},{\"question\":\"What differences were observed between low-probability and intermediate-to-high probability PH groups?\",\"answer\":\"The intermediate-to-high probability group had higher dermato-specific antibody prevalence, lower FVC and DLCO, and higher all-cause mortality and lung transplantation rates.\"},{\"question\":\"Did immunosuppression status change mortality outcomes in the intermediate-to-high probability group?\",\"answer\":\"No significant difference in all-cause mortality was found between patients on immunosuppression versus those not on immunosuppression within the intermediate-to-high probability PH group.\"}]","Echocardiographic Pulmonary Hypertension in Patients Positive for Myositis-Specific and Myositis-Associated Antibodies | PDF",1790761756,33]