{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,16]],"date-time":"2026-10-16T01:55:27Z","timestamp":1792115727670,"version":"4.4.0"},"reference-count":114,"publisher":"MDPI AG","issue":"13","license":[{"start":{"date-parts":[[2025,7,4]],"date-time":"2025-07-04T00:00:00Z","timestamp":1751587200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Pulmonary arterial hypertension (PAH) is a severe complication associated with connective tissue diseases (CTDs), which is characterized by a significant influence on the patient\u2019s prognosis and mortality. The prevalence of PAH varies depending on the type of CTD. Still, it is highly prevalent in patients with systemic sclerosis (SSc), systemic lupus erythematosus (SLE), mixed connective tissue disease (MCTD), and primary Sj\u00f6gren\u2019s syndrome (pSS). Identifying rheumatic disease-specific risk factors is crucial for early diagnosis and intervention. Risk factors for PAH development include specific sociological factors (related to race, gender, and age), clinical features (particularly severe Raynaud\u2019s phenomenon and multiple telangiectasias), cardiological factors (pericarditis and left heart disease), biochemical factors (elevated NT-proBNP and decreased HDL-cholesterol), serological factors (presence of ANA, e.g., anti-U1-RNP or SSA, and antiphospholipid antibodies), and pulmonary factors (interstitial lung disease and decreased DLCO or DLCO\/alveolar volume ratio &lt; 70%, FVC\/DLCO &gt; 1.6). The analysis of risk factors can be the most useful during the selection of patients at high risk of PAH development. The initial diagnosis of PAH is usually based on transthoracic echocardiography (TTE) and is finally confirmed by right heart catheterization (RHC). Targeted therapies can improve outcomes and include endothelin receptor antagonists, prostacyclin analogs, phosphodiesterase inhibitors, and tailored immunosuppressive treatments. Effective management strategies require a multidisciplinary approach involving rheumatologists, cardiologists, and pulmonologists. The risk stratification and individualized treatment strategies can enhance survival and quality of life in patients with PAH-CTD.<\/jats:p>","DOI":"10.3390\/jcm14134742","type":"journal-article","created":{"date-parts":[[2025,7,7]],"date-time":"2025-07-07T06:03:13Z","timestamp":1751868193000},"page":"4742","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":3,"title":["Insights into Pulmonary Arterial Hypertension in Connective Tissue Diseases"],"prefix":"10.3390","volume":"14","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3438-0764","authenticated-orcid":false,"given":"Bogna","family":"Grygiel-G\u00f3rniak","sequence":"first","affiliation":[{"name":"Department of Rheumatology, Rehabilitation and Internal Diseases, Poznan University of Medical Sciences, 61-701 Pozna\u0144, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mateusz","family":"Lucki","sequence":"additional","affiliation":[{"name":"Department and Clinic of Cardiology, University of Medical Sciences, 60-545 Pozna\u0144, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Przemys\u0142aw","family":"Daroszewski","sequence":"additional","affiliation":[{"name":"Department of Organization and Management in Healthcare, Pozna\u0144 University of Medical Sciences, 60-545 Pozna\u0144, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-8248-2247","authenticated-orcid":false,"given":"Ewa","family":"Lucka","sequence":"additional","affiliation":[{"name":"Clinical Rehabilitation Laboratory, Department of Rehabilitation and Physiotherapy, University of Medical Sciences, 60-545 Pozna\u0144, Poland"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2025,7,4]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","unstructured":"Giuc\u0103, A., Mihai, C., Jurcu\u021b, C., Gheorghiu, A.M., Gro\u0219eanu, L., Dima, A., S\u0103ftoiu, A., Coman, I.M., Popescu, B.A., and Jurcu\u021b, R. (2021). Screening for Pulmonary Hypertension in Systemic Sclerosis-A Primer for Cardio-Rheumatology Clinics. Diagnostics, 11.","DOI":"10.3390\/diagnostics11061013"},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"98","DOI":"10.3899\/jrheum.090697","article-title":"Telangiectases in scleroderma: A potential clinical marker of pulmonary arterial hypertension","volume":"37","author":"Shah","year":"2010","journal-title":"J. Rheumatol."},{"key":"ref_3","doi-asserted-by":"crossref","unstructured":"Sato, T., Hatano, M., Iwasaki, Y., Maki, H., Saito, A., Minatsuki, S., Inaba, T., Amiya, E., Fujio, K., and Watanabe, M. (2018). Prevalence of primary Sj\u00f6gren\u2019s syndrome in patients undergoing evaluation for pulmonary arterial hypertension. PLoS ONE, 13.","DOI":"10.1371\/journal.pone.0197297"},{"key":"ref_4","doi-asserted-by":"crossref","unstructured":"Parperis, K., Velidakis, N., Khattab, E., Gkougkoudi, E., and Kadoglou, N.P.E. (2023). Systemic Lupus Erythematosus and Pulmonary Hypertension. Int. J. Mol. Sci., 24.","DOI":"10.3390\/ijms24065085"},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"963","DOI":"10.1183\/09031936.00182813","article-title":"Connective tissue disease-associated pulmonary arterial hypertension in Chinese patients","volume":"44","author":"Hao","year":"2014","journal-title":"Eur. Respir. J."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"e000356","DOI":"10.1136\/lupus-2019-000356","article-title":"Autoantibody clustering of lupus-associated pulmonary hypertension","volume":"6","author":"Mizus","year":"2019","journal-title":"Lupus Sci. Med."},{"key":"ref_7","doi-asserted-by":"crossref","first-page":"524","DOI":"10.1111\/1756-185X.12262","article-title":"High levels of uric acid in systemic lupus erythematosus is associated with pulmonary hypertension","volume":"18","author":"Kim","year":"2015","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"1251","DOI":"10.1002\/art.40512","article-title":"Brief report: Lupus\u2014An unrecognized leading cause of death in young females: A population-based study using nationwide death certificates, 2000\u20132015","volume":"70","author":"Yen","year":"2018","journal-title":"Arthritis Rheumatol."},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"1847","DOI":"10.1002\/art.41740","article-title":"Predicting the Risk of Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus: A Chinese Systemic Lupus Erythematosus Treatment and Research Group Cohort Study","volume":"73","author":"Qu","year":"2021","journal-title":"Arthritis Rheumatol."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"e2761","DOI":"10.1097\/MD.0000000000002761","article-title":"Baseline Characteristics and Risk Factors of Pulmonary Arterial Hypertension in Systemic Lupus Erythematosus Patients","volume":"95","author":"Huang","year":"2016","journal-title":"Medicine"},{"key":"ref_11","doi-asserted-by":"crossref","first-page":"756","DOI":"10.1016\/j.autrev.2010.06.005","article-title":"Anti-Scl-70 (topo-I) antibodies in SLE: Myth or reality?","volume":"9","author":"Mahler","year":"2010","journal-title":"Autoimmun. Rev."},{"key":"ref_12","doi-asserted-by":"crossref","first-page":"1619","DOI":"10.1017\/S1047951121000688","article-title":"Risk factors of pulmonary arterial hypertension in patients with systemic lupus erythematosus","volume":"31","author":"Lei","year":"2021","journal-title":"Cardiol. Young"},{"key":"ref_13","doi-asserted-by":"crossref","first-page":"419","DOI":"10.2332\/allergolint.11-RAI-0362","article-title":"European Society of Cardiology, European Respiratory Society 2009 ESC\/ERS pulmonary hypertension guidelines and connective tissue disease","volume":"60","author":"Nakanishi","year":"2011","journal-title":"Allergol. Int."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"2250","DOI":"10.1161\/CIRCULATIONAHA.109.192230","article-title":"ACCF\/AHA 2009 expert consensus document on pulmonary hypertension: A report of the American College of Cardiology Foundation Task Force on Expert Consensus Documents and the American Heart Association: Developed in collaboration with the American College of Chest Physicians, American Thoracic Society, Inc., and the Pulmonary Hypertension Association","volume":"119","author":"McLaughlin","year":"2009","journal-title":"Circulation"},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"3618","DOI":"10.1093\/eurheartj\/ehac237","article-title":"ESC\/ERS Scientific Document Group. 2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"43","author":"Humbert","year":"2022","journal-title":"Eur. Heart J."},{"key":"ref_16","doi-asserted-by":"crossref","first-page":"1139","DOI":"10.1093\/rheumatology\/kead360","article-title":"Treatment strategies and survival of patients with connective tissue disease and pulmonary arterial hypertension: A COMPERA analysis","volume":"63","author":"Distler","year":"2024","journal-title":"Rheumatology"},{"key":"ref_17","doi-asserted-by":"crossref","first-page":"2290","DOI":"10.3899\/jrheum.100245","article-title":"Prevalence of pulmonary hypertension in systemic sclerosis in European Caucasians and metaanalysis of 5 studies","volume":"37","author":"Avouac","year":"2010","journal-title":"J. Rheumatol."},{"key":"ref_18","doi-asserted-by":"crossref","first-page":"1085","DOI":"10.1177\/0961203314527366","article-title":"Chinese SLE Treatment and Research group (CSTAR) registry: II. Prevalence and risk factors of pulmonary arterial hypertension in Chinese patients with systemic lupus erythematosus","volume":"23","author":"Li","year":"2014","journal-title":"Lupus"},{"key":"ref_19","doi-asserted-by":"crossref","first-page":"1211","DOI":"10.1007\/s00296-012-2525-y","article-title":"Pulmonary hypertension in systemic lupus erythematosus: A systematic review and analysis of 642 cases in Chinese population","volume":"33","author":"Xia","year":"2013","journal-title":"Rheumatol. Int."},{"key":"ref_20","doi-asserted-by":"crossref","first-page":"1846","DOI":"10.1093\/rheumatology\/kes140","article-title":"Clinical characteristics and survival of Japanese patients with connective tissue disease and pulmonary arterial hypertension: A single-centre cohort","volume":"51","author":"Shirai","year":"2012","journal-title":"Rheumatology"},{"key":"ref_21","doi-asserted-by":"crossref","first-page":"1211","DOI":"10.1378\/chest.08-3042","article-title":"French PAH-SSc Network. Is pulmonary arterial hypertension really a late complication of systemic sclerosis?","volume":"136","author":"Hachulla","year":"2009","journal-title":"Chest"},{"key":"ref_22","doi-asserted-by":"crossref","first-page":"2172","DOI":"10.3899\/jrheum.101243","article-title":"Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma (PHAROS): Baseline characteristics and description of study population","volume":"38","author":"Hinchcliff","year":"2011","journal-title":"J. Rheumatol."},{"key":"ref_23","first-page":"S673","article-title":"Better survival in systemic sclerosis-associated pulmonary arterial hypertension patients enrolled in the Pulmonary Hypertension Assessment and Recognition of Outcomes in Scleroderma Registry (PHAROS)","volume":"63","author":"Chung","year":"2011","journal-title":"Arthritis Rheum."},{"key":"ref_24","doi-asserted-by":"crossref","unstructured":"Morrisroe, K., Huq, M., Stevens, W., Rabusa, C., Proudman, S.M., and Nikpour, M. (2016). Australian Scleroderma Interest Group (ASIG). Risk factors for development of pulmonary arterial hypertension in Australian systemic sclerosis patients: Results from a large multicenter cohort study. BMC Pulm. Med., 16.","DOI":"10.1186\/s12890-016-0296-z"},{"key":"ref_25","unstructured":"Kolton Schneider, L., Ali, Y.S., and Agnew, J.D. (2025). Scleroderma-Associated Pulmonary Arterial Hypertension: Early Detection for Better Outcomes (Archived). StatPearls [Internet], StatPearls Publishing."},{"key":"ref_26","doi-asserted-by":"crossref","first-page":"R143","DOI":"10.1186\/ar3876","article-title":"N-terminal pro-brain natriuretic peptide in a novel screening algorithm for pulmonary arterial hypertension in systemic sclerosis: A case-control study","volume":"14","author":"Thakkar","year":"2012","journal-title":"Arthritis Res. Ther."},{"key":"ref_27","doi-asserted-by":"crossref","first-page":"373","DOI":"10.1016\/j.ijcard.2014.02.042","article-title":"Determinants of exercise-induced pulmonary arterial hypertension in systemic sclerosis","volume":"173","author":"Voilliot","year":"2014","journal-title":"Int. J. Cardiol."},{"key":"ref_28","doi-asserted-by":"crossref","first-page":"410","DOI":"10.1016\/j.autrev.2012.07.010","article-title":"Pulmonary hypertension in systemic lupus erythematosus: Prevalence, predictors and diagnostic strategy","volume":"12","author":"Garmendia","year":"2013","journal-title":"Autoimmun. Rev."},{"key":"ref_29","doi-asserted-by":"crossref","first-page":"1506","DOI":"10.1093\/rheumatology\/kep203","article-title":"Prevalence and risk factors for pulmonary arterial hypertension in patients with lupus","volume":"48","author":"Prabu","year":"2009","journal-title":"Rheumatology"},{"key":"ref_30","doi-asserted-by":"crossref","first-page":"3261","DOI":"10.1093\/rheumatology\/kead055","article-title":"In-depth characterization of pulmonary arterial hypertension in mixed connective tissue disease: A French national multicentre study","volume":"62","author":"Chaigne","year":"2023","journal-title":"Rheumatology"},{"key":"ref_31","first-page":"2301","article-title":"The prevalence of pulmonary arterial hypertension in patients with mixed connective tissue disease: A systematic review and meta-analysis","volume":"41","author":"Hassan","year":"2023","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_32","doi-asserted-by":"crossref","first-page":"1134","DOI":"10.3899\/jrheum.121272","article-title":"Clinical course, prognosis, and causes of death in mixed connective tissue disease","volume":"40","author":"Hajas","year":"2013","journal-title":"J. Rheumatol."},{"key":"ref_33","doi-asserted-by":"crossref","first-page":"284","DOI":"10.1097\/BOR.0000000000000787","article-title":"Interstitial lung disease throughout the rheumatoid arthritis disease course","volume":"33","author":"McDermott","year":"2021","journal-title":"Curr. Opin. Rheumatol."},{"key":"ref_34","doi-asserted-by":"crossref","first-page":"173","DOI":"10.1055\/s-0039-1685214","article-title":"Pulmonary Hypertension Associated with Connective Tissue Disease","volume":"40","author":"Fayed","year":"2019","journal-title":"Semin. Respir. Crit. Care Med."},{"key":"ref_35","first-page":"164","article-title":"Rheumatoid arthritis associated pulmonary hypertension: Clinical challenges reflecting the diversity of pathophysiology","volume":"20","author":"Panagiotidou","year":"2017","journal-title":"Respir. Med. Case Rep."},{"key":"ref_36","doi-asserted-by":"crossref","first-page":"48","DOI":"10.1111\/resp.12464","article-title":"Survival in rheumatoid arthritis-associated pulmonary arterial hypertension compared with idiopathic pulmonary arterial hypertension","volume":"20","author":"Sadeghi","year":"2015","journal-title":"Respirology"},{"key":"ref_37","doi-asserted-by":"crossref","first-page":"2401325","DOI":"10.1183\/13993003.01325-2024","article-title":"Treatment algorithm for pulmonary arterial hypertension","volume":"64","author":"Chin","year":"2024","journal-title":"Eur. Respir. J."},{"key":"ref_38","doi-asserted-by":"crossref","first-page":"2651","DOI":"10.1007\/s10067-020-05544-z","article-title":"Echocardiographic evaluation of pulmonary hypertension, right ventricular function, and right ventricular-pulmonary arterial coupling in patients with rheumatoid arthritis","volume":"40","year":"2021","journal-title":"Clin. Rheumatol."},{"key":"ref_39","doi-asserted-by":"crossref","unstructured":"Szturmowicz, M., Franczuk, M., J\u0119drych, M.E., Wyrostkiewicz, D., Oniszh, K., Darocha, S., Kasperowicz, K., and Kurzyna, M. (2021). Dominating Cause of Pulmonary Hypertension May Change Over Time-Diagnostic and Therapeutic Considerations in a Patient with Pulmonary Hypertension Due to Rheumatoid Arthritis with Lung Involvement. Diagnostics, 11.","DOI":"10.3390\/diagnostics11101931"},{"key":"ref_40","doi-asserted-by":"crossref","first-page":"1","DOI":"10.1183\/09059180.00008014","article-title":"Rheumatoid arthritis-associated lung disease","volume":"24","author":"Shaw","year":"2015","journal-title":"Eur. Respir. Rev."},{"key":"ref_41","doi-asserted-by":"crossref","first-page":"295","DOI":"10.1002\/art.41512","article-title":"Hemodynamic Response to Treatment and Outcomes in Pulmonary Hypertension Associated with Interstitial Lung Disease Versus Pulmonary Arterial Hypertension in Systemic Sclerosis: Data from a Study Identifying Prognostic Factors in Pulmonary Hypertension Associated with Interstitial Lung Disease","volume":"73","author":"Chauvelot","year":"2021","journal-title":"Arthritis Rheumatol."},{"key":"ref_42","doi-asserted-by":"crossref","first-page":"337","DOI":"10.1016\/j.chest.2020.08.2069","article-title":"Development and Validation of an Abridged Version of the REVEAL 2.0 Risk Score Calculator, REVEAL Lite 2, for Use in Patients with Pulmonary Arterial Hypertension","volume":"159","author":"Benza","year":"2021","journal-title":"Chest"},{"key":"ref_43","first-page":"3252","article-title":"Clinical and prognostic characteristics of 573 cases of primary Sj\u00f6gren\u2019s syndrome","volume":"123","author":"Lin","year":"2010","journal-title":"Chin. Med. J."},{"key":"ref_44","doi-asserted-by":"crossref","first-page":"1767","DOI":"10.2169\/internalmedicine.8095-21","article-title":"Successful Early Immunosuppressive Therapy for Pulmonary Arterial Hypertension Due to Takayasu arteritis: Two Case Reports and a Review of Similar Case Reports in the English Literature","volume":"61","author":"Suda","year":"2022","journal-title":"Intern. Med."},{"key":"ref_45","doi-asserted-by":"crossref","first-page":"563","DOI":"10.55730\/1300-0144.5617","article-title":"Pulmonary hypertension in Beh\u00e7et\u2019s disease: Echocardiographic screening and multidisciplinary approach","volume":"53","author":"Erden","year":"2023","journal-title":"Turk. J. Med. Sci."},{"key":"ref_46","first-page":"e2023020","article-title":"Pulmonary hypertension in ANCA-associated vasculitis: A retrospective analysis","volume":"40","author":"Baqir","year":"2023","journal-title":"Sarcoidosis Vasc. Diffus. Lung Dis."},{"key":"ref_47","first-page":"710401","article-title":"Pulmonary Arterial Hypertension in Patients with Primary Sj\u00f6gren\u2019s Syndrome","volume":"2014","author":"Kobak","year":"2014","journal-title":"Autoimmune Dis."},{"key":"ref_48","doi-asserted-by":"crossref","first-page":"516","DOI":"10.1002\/art.10775","article-title":"Predictors of isolated pulmonary hypertension in patients with systemic sclerosis and limited cutaneous involvement","volume":"48","author":"Steen","year":"2003","journal-title":"Arthritis Rheum."},{"key":"ref_49","doi-asserted-by":"crossref","first-page":"893","DOI":"10.1016\/j.humpath.2006.11.022","article-title":"Fibrous remodeling of the pulmonary venous system in pulmonary arterial hypertension associated with connective tissue diseases","volume":"38","author":"Humbert","year":"2007","journal-title":"Hum. Pathol."},{"key":"ref_50","doi-asserted-by":"crossref","first-page":"92","DOI":"10.1016\/j.chest.2017.02.010","article-title":"Idiopathic and Systemic Sclerosis-Associated Pulmonary Arterial Hypertension: A Comparison of Demographic, Hemodynamic, and MRI Characteristics and Outcomes","volume":"152","author":"Ramjug","year":"2017","journal-title":"Chest"},{"key":"ref_51","doi-asserted-by":"crossref","first-page":"1423","DOI":"10.1093\/cvr\/cvx145","article-title":"RV pressure overload: From hypertrophy to failure","volume":"113","author":"Tedford","year":"2017","journal-title":"Cardiovasc. Res."},{"key":"ref_52","doi-asserted-by":"crossref","first-page":"210053","DOI":"10.1183\/16000617.0053-2021","article-title":"Pulmonary hypertension phenotypes in patients with systemic sclerosis","volume":"30","author":"Haque","year":"2021","journal-title":"Eur. Respir. Rev."},{"key":"ref_53","doi-asserted-by":"crossref","unstructured":"Tu, J., Jin, J., Chen, X., Sun, L., and Cai, Z. (2022). Altered Cellular Immunity and Differentially Expressed Immune-Related Genes in Patients with Systemic Sclerosis-Associated Pulmonary Arterial Hypertension. Front. Immunol., 13.","DOI":"10.3389\/fimmu.2022.868983"},{"key":"ref_54","doi-asserted-by":"crossref","first-page":"10","DOI":"10.1186\/s13075-020-02383-w","article-title":"Lymphocyte Subset Abnormalities in Early Diffuse Cutaneous Systemic Sclerosis","volume":"23","author":"Fox","year":"2021","journal-title":"Arthritis Res. Ther."},{"key":"ref_55","doi-asserted-by":"crossref","first-page":"169","DOI":"10.1007\/s00011-017-1106-7","article-title":"Increased frequencies of circulating CXCL10-, CXCL8- and CCL4-producing monocytes and Siglec-3-expressing myeloid dendritic cells in systemic sclerosis patients","volume":"67","author":"Carvalheiro","year":"2018","journal-title":"Inflamm. Res."},{"key":"ref_56","doi-asserted-by":"crossref","first-page":"1539","DOI":"10.1136\/ard.2009.128207","article-title":"Distinct evolution of TLR-mediated dendritic cell cytokine secretion in patients with limited and diffuse cutaneous systemic sclerosis","volume":"69","author":"Popa","year":"2010","journal-title":"Ann. Rheum. Dis."},{"key":"ref_57","doi-asserted-by":"crossref","unstructured":"Pendergrass, S.A., Hayes, E., Farina, G., Lemaire, R., Farber, H.W., Whitfield, M.L., and Lafyatis, R. (2010). Limited systemic sclerosis patients with pulmonary arterial hypertension show biomarkers of inflammation and vascular injury. PLoS ONE, 5.","DOI":"10.1371\/journal.pone.0012106"},{"key":"ref_58","doi-asserted-by":"crossref","first-page":"6086","DOI":"10.1111\/jocd.15133","article-title":"Peripheral blood IL-6 levels in systemic sclerosis patients: Correlation between IL-6 levels and clinical phenotypes","volume":"21","author":"Lin","year":"2022","journal-title":"J. Cosmet. Dermatol."},{"key":"ref_59","doi-asserted-by":"crossref","unstructured":"Cardoneanu, A., Burlui, A.M., Macovei, L.A., Bratoiu, I., Richter, P., and Rezus, E. (2022). Targeting Systemic Sclerosis from Pathogenic Mechanisms to Clinical Manifestations: Why IL-6?. Biomedicines, 10.","DOI":"10.3390\/biomedicines10020318"},{"key":"ref_60","doi-asserted-by":"crossref","first-page":"95","DOI":"10.1183\/09031936.00074309","article-title":"Disproportionate elevation of N-terminal pro-brain natriuretic peptide in scleroderma-related pulmonary hypertension","volume":"35","author":"Mathai","year":"2010","journal-title":"Eur. Respir. J."},{"key":"ref_61","doi-asserted-by":"crossref","first-page":"323","DOI":"10.1016\/j.it.2017.02.002","article-title":"PSGL-1, A New Player in the Immune Checkpoint Landscape","volume":"38","author":"Tinoco","year":"2017","journal-title":"Trends Immunol."},{"key":"ref_62","doi-asserted-by":"crossref","first-page":"477","DOI":"10.1002\/art.41100","article-title":"Spontaneous Pulmonary Hypertension Associated with Systemic Sclerosis in P-Selectin Glycoprotein Ligand 1-Deficient Mice","volume":"72","author":"Serrador","year":"2020","journal-title":"Arthritis Rheumatol."},{"key":"ref_63","doi-asserted-by":"crossref","first-page":"e001361","DOI":"10.1136\/lupus-2024-001361","article-title":"Mortality patterns of SLE and the associated risk factors in Korean patients: A nationwide cohort study","volume":"12","author":"Cho","year":"2025","journal-title":"Lupus Sci. Med."},{"key":"ref_64","doi-asserted-by":"crossref","first-page":"411","DOI":"10.2332\/allergolint.11-RAI-0337","article-title":"A histopathological study of pulmonary hypertension in connective tissue disease","volume":"60","author":"Sasaki","year":"2011","journal-title":"Allergol. Int."},{"key":"ref_65","doi-asserted-by":"crossref","first-page":"197","DOI":"10.1186\/s12931-025-03273-y","article-title":"First nomogram for predicting interstitial lung disease and pulmonary arterial hypertension in SLE: A machine learning approach","volume":"26","author":"Niu","year":"2025","journal-title":"Respir. Res."},{"key":"ref_66","first-page":"1","article-title":"Systemic lupus erythematosus and pulmonary arterial hypertension: Links, risks, and management strategies","volume":"9","author":"Tselios","year":"2017","journal-title":"Open Access Rheumatol."},{"key":"ref_67","doi-asserted-by":"crossref","first-page":"349","DOI":"10.1016\/0049-0172(84)90015-5","article-title":"Immunopathologic and clinical studies in pulmonary hypertension associated with systemic lupus erythematosus","volume":"13","author":"Quismorio","year":"1984","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_68","first-page":"409","article-title":"Anti-endothelial cell antibodies in mixed connective tissue disease: Frequency and association with clinical symptoms","volume":"22","author":"Bodolay","year":"2004","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_69","doi-asserted-by":"crossref","first-page":"484","DOI":"10.1002\/art.39432","article-title":"Characteristics and survival of anti-U1 RNP antibody-positive patients with connective tissue disease-associated pulmonary arterial hypertension","volume":"68","author":"Sobanski","year":"2016","journal-title":"Arthritis Rheum."},{"key":"ref_70","doi-asserted-by":"crossref","first-page":"26","DOI":"10.1016\/j.ejrad.2018.08.005","article-title":"Radiological images of interstitial pneumonia in mixed connective tissue disease compared with scleroderma and polymyositis\/dermatomyositis","volume":"107","author":"Yamanaka","year":"2018","journal-title":"Eur. J. Radiol."},{"key":"ref_71","doi-asserted-by":"crossref","first-page":"326","DOI":"10.1016\/j.ijcard.2013.12.079","article-title":"Cardiac involvement in mixed connective tissue disease: A systematic review","volume":"171","author":"Ungprasert","year":"2014","journal-title":"Int. J. Cardiol."},{"key":"ref_72","doi-asserted-by":"crossref","first-page":"957","DOI":"10.1016\/j.semarthrit.2020.06.005","article-title":"Epidemiology and mortality of connective tissue disease-associated pulmonary arterial hypertension: A national cohort study in Taiwan","volume":"50","author":"Lin","year":"2020","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_73","doi-asserted-by":"crossref","unstructured":"Chen, J., Norling, L.V., and Cooper, D. (2021). Cardiac dysfunction in rheumatoid arthritis: The role of inflammation. Cells, 10.","DOI":"10.3390\/cells10040881"},{"key":"ref_74","doi-asserted-by":"crossref","first-page":"1211","DOI":"10.1007\/s10067-020-05320-z","article-title":"Rheumatoid arthritis-associated interstitial lung disease: An overview of epidemiology, pathogenesis and management","volume":"40","author":"Dai","year":"2021","journal-title":"Clin. Rheumatol."},{"key":"ref_75","doi-asserted-by":"crossref","first-page":"2957","DOI":"10.1161\/01.CIR.0000099844.31524.05","article-title":"Explaining how \u201chigh-grade\u201d systemic inflammation accelerates vascular risk in rheumatoid arthritis","volume":"108","author":"Sattar","year":"2003","journal-title":"Circulation"},{"key":"ref_76","doi-asserted-by":"crossref","first-page":"96","DOI":"10.1007\/s00059-019-4785-8","article-title":"Key inflammatory mechanisms underlying heart failure","volume":"44","author":"Riehle","year":"2019","journal-title":"Herz"},{"key":"ref_77","doi-asserted-by":"crossref","first-page":"263","DOI":"10.1016\/j.jacc.2013.02.092","article-title":"A novel paradigm for heart failure with preserved ejection fraction: Comorbidities drive myocardial dysfunction and remodeling through coronary microvascular endothelial inflammation","volume":"62","author":"Paulus","year":"2013","journal-title":"J. Am. Coll. Cardiol."},{"key":"ref_78","doi-asserted-by":"crossref","first-page":"e004312","DOI":"10.1161\/CIRCHEARTFAILURE.117.004312","article-title":"Association of Biomarker Clusters with Cardiac Phenotypes and Mortality in Patients with HIV Infection","volume":"11","author":"Scherzer","year":"2018","journal-title":"Circ. Heart Fail."},{"key":"ref_79","doi-asserted-by":"crossref","first-page":"379","DOI":"10.1016\/j.ijcard.2017.03.108","article-title":"Five-year changes in cardiac structure and function in patients with rheumatoid arthritis compared with the general population","volume":"240","author":"Davis","year":"2017","journal-title":"Int. J. Cardiol."},{"key":"ref_80","doi-asserted-by":"crossref","first-page":"44","DOI":"10.1161\/CIRCHEARTFAILURE.109.931451","article-title":"Cardiac inflammation contributes to changes in the extracellular matrix in patients with heart failure and normal ejection fraction","volume":"4","author":"Westermann","year":"2011","journal-title":"Circ. Heart Fail."},{"key":"ref_81","doi-asserted-by":"crossref","first-page":"667","DOI":"10.1002\/art.27264","article-title":"Cardiovascular disease in patients with inflammatory rheumatic disease is associated with up-regulation of markers of inflammation in cardiac microvessels and cardiomyocytes","volume":"62","author":"Grundtman","year":"2010","journal-title":"Arthritis Rheum."},{"key":"ref_82","first-page":"109","article-title":"Is the heart affected in primary Sj\u00f6gren\u2019s syndrome? An echocardiographic study","volume":"26","author":"Vassiliou","year":"2008","journal-title":"Clin. Exp. Rheumatol."},{"key":"ref_83","doi-asserted-by":"crossref","unstructured":"Berardicurti, O., Marino, A., Genovali, I., Navarini, L., D\u2019Andrea, S., Currado, D., Rigon, A., Arcarese, L., Vadacca, M., and Giacomelli, R. (2023). Interstitial Lung Disease and Pulmonary Damage in Primary Sj\u00f6gren\u2019s Syndrome: A Systematic Review and Meta-Analysis. J. Clin. Med., 12.","DOI":"10.3390\/jcm12072586"},{"key":"ref_84","doi-asserted-by":"crossref","first-page":"220210","DOI":"10.1183\/16000617.0210-2022","article-title":"Prevalence, imaging patterns and risk factors of interstitial lung disease in connective tissue disease: A systematic review and meta-analysis","volume":"32","author":"Joy","year":"2023","journal-title":"Eur. Respir. Rev."},{"key":"ref_85","doi-asserted-by":"crossref","first-page":"222","DOI":"10.1016\/j.semarthrit.2017.03.004","article-title":"Incidence, mortality, and causes of death in physician-diagnosed primary Sj\u00f6gren\u2019s syndrome in Korea: A nationwide, population-based study","volume":"47","author":"Kim","year":"2017","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_86","doi-asserted-by":"crossref","unstructured":"Atzeni, F., Gozza, F., Cafaro, G., Perricone, C., and Bartoloni, E. (2022). Cardiovascular Involvement in Sj\u00f6gren\u2019s Syndrome. Front. Immunol., 13.","DOI":"10.3389\/fimmu.2022.879516"},{"key":"ref_87","doi-asserted-by":"crossref","first-page":"299","DOI":"10.1097\/MD.0b013e3181579781","article-title":"Pulmonary arterial hypertension: A rare complication of primary Sj\u00f6gren syndrome: Report of 9 new cases and review of the literature","volume":"86","author":"Launay","year":"2007","journal-title":"Medicine"},{"key":"ref_88","doi-asserted-by":"crossref","first-page":"1902157","DOI":"10.1183\/13993003.02157-2019","article-title":"Pulmonary arterial hypertension associated with primary Sj\u00f6gren\u2019s syndrome: A multicentre cohort study from China","volume":"56","author":"Wang","year":"2020","journal-title":"Eur. Respir. J."},{"key":"ref_89","doi-asserted-by":"crossref","first-page":"e000137","DOI":"10.1136\/bmjmed-2022-000137","article-title":"Pathophysiology and new advances in pulmonary hypertension","volume":"2","author":"Bousseau","year":"2023","journal-title":"BMJ Med."},{"key":"ref_90","doi-asserted-by":"crossref","first-page":"3943","DOI":"10.2174\/138161205774580561","article-title":"Molecular mechanisms of and possible treatment strategies for idiopathic pulmonary fibrosis","volume":"11","author":"Phan","year":"2005","journal-title":"Curr. Pharm. Des."},{"key":"ref_91","doi-asserted-by":"crossref","first-page":"159","DOI":"10.1161\/01.CIR.0000102381.57477.50","article-title":"Endothelial dysfunction in pulmonary hypertension","volume":"109","author":"Budhiraja","year":"2004","journal-title":"Circulation"},{"key":"ref_92","doi-asserted-by":"crossref","unstructured":"Ma, D., Feng, Y., and Lin, X. (2024). Immune and non-immune mediators in the fibrosis pathogenesis of salivary gland in Sj\u00f6gren\u2019s syndrome. Front. Immunol., 15.","DOI":"10.3389\/fimmu.2024.1421436"},{"key":"ref_93","doi-asserted-by":"crossref","unstructured":"Gupta, S., Ferrada, M.A., and Hasni, S.A. (2019). Pulmonary Manifestations of Primary Sj\u00f6gren\u2019s Syndrome: Underlying Immunological Mechanisms, Clinical Presentation, and Management. Front. Immunol., 10.","DOI":"10.3389\/fimmu.2019.01327"},{"key":"ref_94","doi-asserted-by":"crossref","unstructured":"Hinrichs, A.C., Blokland, S.L.M., Kruize, A.A., Lafeber, F.P.J., Leavis, H.L., and van Roon, J.A.G. (2022). CCL5 Release by CCR9+ CD8 T Cells: A Potential Contributor to Immunopathology of Primary Sj\u00f6gren\u2019s Syndrome. Front. Immunol., 13.","DOI":"10.3389\/fimmu.2022.887972"},{"key":"ref_95","doi-asserted-by":"crossref","first-page":"534","DOI":"10.1164\/ajrccm.165.4.2012112","article-title":"Chemokine RANTES in severe pulmonary arterial hypertension","volume":"165","author":"Zarka","year":"2002","journal-title":"Am. J. Respir. Crit. Care Med."},{"key":"ref_96","doi-asserted-by":"crossref","first-page":"293","DOI":"10.1186\/s13075-021-02675-9","article-title":"The clinical features of pulmonary artery involvement in Takayasu arteritis and its relationship with ischemic heart diseases and infection","volume":"23","author":"Mukoyama","year":"2021","journal-title":"Arthritis Res. Ther."},{"key":"ref_97","doi-asserted-by":"crossref","first-page":"61","DOI":"10.1016\/j.autrev.2011.08.001","article-title":"Pathogenesis of Takayasu\u2019s arteritis: A 2011 update","volume":"11","author":"Arnaud","year":"2011","journal-title":"Autoimmun. Rev."},{"key":"ref_98","doi-asserted-by":"crossref","first-page":"495","DOI":"10.3899\/jrheum.140436","article-title":"Takayasu arteritis-associated pulmonary hypertension","volume":"42","author":"Wang","year":"2015","journal-title":"J. Rheumatol."},{"key":"ref_99","doi-asserted-by":"crossref","first-page":"40","DOI":"10.1007\/s12016-007-8064-8","article-title":"The unique features of vasculitis in Beh\u00e7et\u2019s syndrome","volume":"35","author":"Melikoglu","year":"2008","journal-title":"Clin. Rev. Allergy Immunol."},{"key":"ref_100","doi-asserted-by":"crossref","first-page":"867","DOI":"10.1016\/j.amjmed.2004.05.027","article-title":"Pulmonary artery aneurysms in Beh\u00e7et syndrome","volume":"117","author":"Hamuryudan","year":"2004","journal-title":"Am. J. Med."},{"key":"ref_101","doi-asserted-by":"crossref","first-page":"601","DOI":"10.1038\/jid.2011.397","article-title":"Identification of HnRNP-A2\/B1 as a target antigen of anti-endothelial cell IgA antibody in Beh\u00e7et\u2019s disease","volume":"132","author":"Cho","year":"2012","journal-title":"J. Investig. Dermatol."},{"key":"ref_102","doi-asserted-by":"crossref","first-page":"640","DOI":"10.1056\/NEJMra2305712","article-title":"Beh\u00e7et\u2019s Syndrome","volume":"390","author":"Saadoun","year":"2024","journal-title":"N. Engl. J. Med."},{"key":"ref_103","doi-asserted-by":"crossref","first-page":"387","DOI":"10.5603\/CJ.2012.0070","article-title":"Comparison of inhaled nitric oxide and aerosolized iloprost in pulmonary hypertension in children with congenital heart surgery","volume":"19","author":"Kirbas","year":"2012","journal-title":"Cardiol. J."},{"key":"ref_104","first-page":"62","article-title":"The Long-Term Response to Treatment with Calcium Channel Blockers in Patients with Idiopathic Pulmonary Arterial Hypertension","volume":"18","author":"Kiani","year":"2023","journal-title":"J. Tehran Heart Cent."},{"key":"ref_105","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1007\/978-3-642-38664-0_9","article-title":"Endothelin receptor antago-nists","volume":"218","author":"Clozel","year":"2013","journal-title":"Handb. Exp. Pharmacol."},{"key":"ref_106","doi-asserted-by":"crossref","unstructured":"Xing, J., Cao, Y., and Yu, Y. (2017). In vitro micropatterned human pluripotent stem cell test (\u03bcP-HPST) for morphometric-based teratogen screening. Sci. Rep., 7.","DOI":"10.1038\/s41598-017-09178-1"},{"key":"ref_107","doi-asserted-by":"crossref","first-page":"567","DOI":"10.1517\/13543784.2011.565048","article-title":"Riociguat for the treat-ment of pulmonary hypertension","volume":"20","author":"Schermuly","year":"2011","journal-title":"Expert Opin. Investig. Drugs"},{"key":"ref_108","doi-asserted-by":"crossref","unstructured":"Papa, S., Scoccia, G., Serino, G., Adamo, F.I., Jabbour, J.P., Caputo, A., Boromei, M., Filomena, D., Laviola, D., and Maggio, E. (2023). Impact of Parenteral Prostanoids in Pulmonary Arterial Hypertension: The Relevance of Timing. J. Clin. Med., 12.","DOI":"10.3390\/jcm12216840"},{"key":"ref_109","doi-asserted-by":"crossref","first-page":"e13555","DOI":"10.1111\/apha.13555","article-title":"Prostacyclin facilitates vascular smooth muscle cell phenotypic transformation via activating TP receptors when IP receptors are deficient","volume":"231","author":"Li","year":"2021","journal-title":"Acta Physiol."},{"key":"ref_110","doi-asserted-by":"crossref","first-page":"982","DOI":"10.1111\/1756-185X.14368","article-title":"Immunosuppressive therapy in patients with connective tissue disease-associated pulmonary arterial hypertension: A systematic review","volume":"25","author":"Ding","year":"2022","journal-title":"Int. J. Rheum. Dis."},{"key":"ref_111","doi-asserted-by":"crossref","first-page":"108192","DOI":"10.1016\/j.pharmthera.2022.108192","article-title":"The treatment strategy of connective tissue disease associated pulmonary arterial hypertension: Evolving into the future","volume":"239","author":"Zhao","year":"2022","journal-title":"Pharmacol. Ther."},{"key":"ref_112","doi-asserted-by":"crossref","first-page":"57","DOI":"10.1161\/CIRCULATIONAHA.113.004526","article-title":"Anticoagulation and survival in pulmonary arterial hypertension: Results from the Comparative, Prospective Registry of Newly Initiated Therapies for Pulmonary Hy-pertension (COMPERA)","volume":"129","author":"Olsson","year":"2014","journal-title":"Circulation"},{"key":"ref_113","doi-asserted-by":"crossref","first-page":"1900276","DOI":"10.1183\/13993003.002762019","article-title":"Effect of domiciliary oxygen therapy on exercise capacity and quality of life in patients with pulmonary arterial or chronic thromboembolic pulmonary hypertension: A randomised, placebo-controlled trial","volume":"54","author":"Ulrich","year":"2019","journal-title":"Eur. Respir. J."},{"key":"ref_114","doi-asserted-by":"crossref","unstructured":"Sonnweber, T., Nairz, M., Theurl, I., Petzer, V., Tymoszuk, P., Haschka, D., Rieger, E., Kaessmann, B., Deri, M., and Watzinger, K. (2018). The crucial impact of iron deficien-cy definition for the course of precapillary pulmonary hypertension. PLoS ONE, 13.","DOI":"10.1371\/journal.pone.0203396"}],"container-title":["Journal of Clinical Medicine"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.mdpi.com\/2077-0383\/14\/13\/4742\/pdf","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2025,10,9]],"date-time":"2025-10-09T18:04:28Z","timestamp":1760033068000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.mdpi.com\/2077-0383\/14\/13\/4742"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2025,7,4]]},"references-count":114,"journal-issue":{"issue":"13","published-online":{"date-parts":[[2025,7]]}},"alternative-id":["jcm14134742"],"URL":"https:\/\/doi.org\/10.3390\/jcm14134742","relation":{},"ISSN":["2077-0383"],"issn-type":[{"value":"2077-0383","type":"electronic"}],"subject":[],"published":{"date-parts":[[2025,7,4]]}}}