{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,21]],"date-time":"2026-07-21T11:13:39Z","timestamp":1784632419436,"version":"3.55.0"},"reference-count":15,"publisher":"MDPI AG","issue":"14","license":[{"start":{"date-parts":[[2026,7,19]],"date-time":"2026-07-19T00:00:00Z","timestamp":1784419200000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Johnson and Johnson Pty Ltd., Sydney, Australia","award":["GA-0664"],"award-info":[{"award-number":["GA-0664"]}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Diagnostics"],"abstract":"<jats:p>Background\/Objectives: Pulmonary arterial hypertension (PAH) occurs in &gt;12% of systemic sclerosis (SSc) patients and has a high mortality. Annual screening for all SSc patients is the standard of care and can include spirometry (RFT), serum N-terminal pro-B-type natriuretic peptide (NT-proBNP) and echocardiography (TTE). We sought to evaluate the accuracy of NT-proBNP alone to screen for SSc-PAH. Methods: We defined groups as low, intermediate and high risk of PAH according to RFT, TTE and RHC (right heart catheter) parameters. NT-proBNP thresholds predictive of risk group were defined, and ROC curves were used to evaluate the predictive accuracy of the calculated cut-points to diagnose SSc-PAH within the following 12 months. Results: A total of 820 SSc patients had NT-proBNP recorded 12 months prior to RHC, or if no RHC had been performed, within 12 months of the most recently recorded annual visit. The majority (335, 40.85%) were assigned to low risk of PAH, 189 (23.05%) to intermediate risk and 176 (21.46%) to high risk. NT-proBNP level &lt; 122.9 ng\/L defined the low-risk group, 122.9\u2013186.6 ng\/L defined the intermediate risk and &gt;186.6 ng\/L defined the high risk. NT-proBNP \u2265210 ng\/L with RFT (the \u2018ASIG algorithm\u2019) had the highest predictive accuracy, with sensitivity of 86.75% (77.52\u201393.19%), specificity of 60.71% (58.52\u201362.87%), PPV of 8.47% (6.69\u201310.55%), NPV of 99.09% (98.38\u201399.55%) and AUC of 0.74 (0.70\u20130.78). NT-proBNP \u2265 210 ng\/L alone had sensitivity of 67.05% (56.21\u201376.70), specificity of 71.91% (70.03\u201373.72), PPV of 8.25% (6.34\u201310.52), NPV of 98.30% (97.57\u201398.86) and AUC of 0.69 (0.64\u20130.74). NT-proBNP thresholds associated with the three defined risk groups performed similarly overall. Conclusions: NT-proBNP alone, compared to the ASIG algorithm, had a slight reduction in NPV and AUC to predict PAH within 12 months. When NT-proBNP is \u2265210 ng\/L, the NPV remains high (98.0%).<\/jats:p>","DOI":"10.3390\/diagnostics16142254","type":"journal-article","created":{"date-parts":[[2026,7,20]],"date-time":"2026-07-20T02:32:08Z","timestamp":1784514728000},"page":"2254","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Predictive Accuracy of Serum N-Terminal Pro-B-Type Natriuretic Peptide Alone and in Combination with Respiratory Function Tests to Identify Systemic Sclerosis-Associated Pulmonary Arterial Hypertension (SSc-PAH)"],"prefix":"10.3390","volume":"16","author":[{"given":"Zoe","family":"Brown","sequence":"first","affiliation":[{"name":"Department of Rheumatology, St Vincent\u2019s Hospital, Melbourne, VIC 3065, Australia"},{"name":"Department of Medicine at St Vincent\u2019s Hospital, The University of Melbourne, Melbourne, VIC 3065, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Dylan","family":"Hansen","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, St Vincent\u2019s Hospital, Melbourne, VIC 3065, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-4636-729X","authenticated-orcid":false,"given":"Laura","family":"Ross","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, St Vincent\u2019s Hospital, Melbourne, VIC 3065, Australia"},{"name":"Department of Medicine at St Vincent\u2019s Hospital, The University of Melbourne, Melbourne, VIC 3065, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Wendy","family":"Stevens","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, St Vincent\u2019s Hospital, Melbourne, VIC 3065, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kathleen","family":"Morrisroe","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, St Vincent\u2019s Hospital, Melbourne, VIC 3065, Australia"},{"name":"Department of Medicine at St Vincent\u2019s Hospital, The University of Melbourne, Melbourne, VIC 3065, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alannah","family":"Quinlivan","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, St Vincent\u2019s Hospital, Melbourne, VIC 3065, Australia"},{"name":"Department of Medicine at St Vincent\u2019s Hospital, The University of Melbourne, Melbourne, VIC 3065, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maryam","family":"Tabesh","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, St Vincent\u2019s Hospital, Melbourne, VIC 3065, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gene-Siew","family":"Ngian","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Monash Health, Clayton, VIC 3168, Australia"},{"name":"Department of Medicine, Monash University, Clayton, VIC 3168, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Diane","family":"Apostolopoulos","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Monash Health, Clayton, VIC 3168, Australia"},{"name":"Department of Medicine, Monash University, Clayton, VIC 3168, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Joanne","family":"Sahhar","sequence":"additional","affiliation":[{"name":"Department of Rheumatology, Monash Health, Clayton, VIC 3168, Australia"},{"name":"Department of Medicine, Monash University, Clayton, VIC 3168, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jennifer G.","family":"Walker","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Flinders Medical Centre, Bedford Park, SA 5042, Australia"},{"name":"Immunology, Allergy and Arthritis Department, Flinders University, Bedford Park, SA 5042, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3480-7251","authenticated-orcid":false,"given":"Lauren","family":"Host","sequence":"additional","affiliation":[{"name":"Fiona Stanley Hospital, Perth, WA 6150, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Susanna","family":"Proudman","sequence":"additional","affiliation":[{"name":"Rheumatology Unit, Royal Adelaide Hospital, Adelaide, SA 5000, Australia"},{"name":"Department of Medicine, The University of Adelaide, Adelaide, SA 5000, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Mandana","family":"Nikpour","sequence":"additional","affiliation":[{"name":"Sydney Musculoskeletal Research Centre and School of Public Health, University of Sydney, Sydney, NSW 2050, Australia"},{"name":"Department of Rheumatology, Royal Prince Alfred Hospital, Sydney, NSW 2050, Australia"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2026,7,19]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"1067","DOI":"10.1002\/art.40027","article-title":"Early Mortality in a Multinational Systemic Sclerosis Inception Cohort","volume":"69","author":"Hao","year":"2017","journal-title":"Arthritis Rheum."},{"key":"ref_2","doi-asserted-by":"crossref","first-page":"3522","DOI":"10.1002\/art.30541","article-title":"Screening for pulmonary arterial hypertension in patients with systemic sclerosis: Clinical characteristics at diagnosis and long-term survival","volume":"63","author":"Humbert","year":"2011","journal-title":"Arthritis Rheum."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"42","DOI":"10.1186\/s13075-017-1250-z","article-title":"Epidemiology and disease characteristics of systemic sclerosis-related pulmonary arterial hypertension: Results from a real-life screening programme","volume":"19","author":"Morrisroe","year":"2017","journal-title":"Arthritis Res. Ther."},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"495","DOI":"10.1016\/j.semarthrit.2021.03.011","article-title":"Screening for the early detection of pulmonary arterial hypertension in patients with systemic sclerosis: A systematic review and meta-analysis of long-term outcomes","volume":"51","author":"Brown","year":"2021","journal-title":"Semin. Arthritis Rheum."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"2200879","DOI":"10.1183\/13993003.00879-2022","article-title":"2022 ESC\/ERS Guidelines for the diagnosis and treatment of pulmonary hypertension","volume":"61","author":"Humbert","year":"2022","journal-title":"Eur. Respir. J."},{"key":"ref_6","doi-asserted-by":"crossref","first-page":"7","DOI":"10.1186\/s13075-015-0517-5","article-title":"A comparison of the predictive accuracy of three screening models for pulmonary arterial hypertension in systemic sclerosis","volume":"17","author":"Hao","year":"2015","journal-title":"Arthritis Res. Ther."},{"key":"ref_7","doi-asserted-by":"crossref","unstructured":"Coirier, V., Chabanne, C., Jouneau, S., Belhomme, N., Ballerie, A., Cazalets, C., Sobanski, V., Hachulla, \u00c9., J\u00e9go, P., and Lescoat, A. (2021). Impact of Three Different Algorithms for the Screening of SSc-PAH and Comparison with the Decisions of a Multidisciplinary Team. Diagnostics, 11.","DOI":"10.3390\/diagnostics11101738"},{"key":"ref_8","doi-asserted-by":"crossref","first-page":"38","DOI":"10.1378\/chest.08-0013","article-title":"Geographic Variation of Spirometry Use in Newly Diagnosed COPD","volume":"134","author":"Joo","year":"2008","journal-title":"Chest"},{"key":"ref_9","doi-asserted-by":"crossref","first-page":"00602-2021","DOI":"10.1183\/23120541.00602-2021","article-title":"International consensus on lung function testing during the COVID-19 pandemic and beyond","volume":"8","author":"McGowan","year":"2022","journal-title":"ERJ Open Res."},{"key":"ref_10","doi-asserted-by":"crossref","first-page":"2737","DOI":"10.1002\/art.38098","article-title":"2013 classification criteria for systemic sclerosis: An American College of Rheumatology\/European League against Rheumatism collaborative initiative","volume":"65","author":"Khanna","year":"2013","journal-title":"Arthritis Rheum."},{"key":"ref_11","first-page":"328","article-title":"Comparison between 3 diagnostic criteria for mixed connective tissue disease. Study of 593 patients","volume":"16","author":"Cardiel","year":"1989","journal-title":"J. Rheumatol."},{"key":"ref_12","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_13","doi-asserted-by":"crossref","first-page":"2676","DOI":"10.1002\/sim.4509","article-title":"Classification accuracy and cut point\u2009selection","volume":"31","author":"Liu","year":"2012","journal-title":"Stat. Med."},{"key":"ref_14","doi-asserted-by":"crossref","first-page":"1340","DOI":"10.1136\/annrheumdis-2013-203301","article-title":"Evidence-based detection of pulmonary arterial hypertension in systemic sclerosis: The DETECT study","volume":"73","author":"Coghlan","year":"2014","journal-title":"Ann. Rheum. Dis."},{"key":"ref_15","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1016\/j.ejim.2020.05.042","article-title":"Screening for pulmonary arterial hypertension in systemic sclerosis: A systematic literature review","volume":"78","author":"Bruni","year":"2020","journal-title":"Eur. J. Intern. 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