{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,7,17]],"date-time":"2026-07-17T14:38:10Z","timestamp":1784299090590,"version":"3.55.0"},"reference-count":20,"publisher":"The Journal of Rheumatology","issue":"4","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["J Rheumatol"],"accepted":{"date-parts":[[2025,11,18]]},"published-print":{"date-parts":[[2026,4]]},"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>Idiopathic pulmonary fibrosis (IPF) and systemic autoimmune rheumatic disease (SARD)-associated interstitial lung disease (ILD) are lung disorders with distinct clinical trajectories. This study aimed to compare survival and pulmonary function trends between IPF and SARD-ILD.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>We retrospectively analyzed 410 patients with ILD (154 IPF, 256 SARD-ILD) from 6 Italian centers. SARD-ILD subtypes included antisynthetase syndrome (ASyS; n = 58), dermatomyositis (DM; n = 55), systemic sclerosis (SSc; n = 106), and Sj\u00f6gren disease (SjD, n = 37). Outcomes included 5-year survival and pulmonary function test (PFT) changes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      Five-year survival was lower in patients with IPF (mean 33.6 months) than in those with SARD-ILD (mean 56.0 months;\n                      <jats:italic>P<\/jats:italic>\n                      &lt; 0.001). SARD subtypes showed comparable survival: 58.2 months in patients with ASyS, 52.9 months in DM, 55.2 months in SSc, and 58.6 months in SjD. Patients with ASyS and DM demonstrated significant functional improvement, with forced vital capacity (FVC) increasing from 71% to 81% in ASyS (+14.1% relative) and from 69% to 78% in DM (+13%). IPF FVC declined from 78% to 72% (\u22127.7%). Usual interstitial pneumonia pattern was universal in IPF but seen in &lt; 20% of patients with SARD-ILD. ILD pattern did not significantly influence functional trajectory in patients with SARD-ILD; instead, diagnosis was the primary determinant (multivariable ANOVA\n                      <jats:italic>P<\/jats:italic>\n                      &lt; 0.001). Multivariable analysis confirmed SARD-ILD as a favorable prognostic factor (adjusted hazard ratio [aHR] 0.21), with age (aHR 1.06) and male sex (aHR 1.98) linked to poorer outcomes.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>SARD-ILD is associated with higher survival than IPF. Functional trajectories improved in patients with ASyS and DM, in contrast to the decline observed in those with IPF. Prognosis is more strongly influenced by the underlying diagnosis, supporting a diagnosis-centered approach to disease management.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.3899\/jrheum.2025-0610","type":"journal-article","created":{"date-parts":[[2026,2,2]],"date-time":"2026-02-02T12:05:15Z","timestamp":1770033915000},"page":"450-455","source":"Crossref","is-referenced-by-count":1,"title":["Prognostic and Functional Trajectories in Idiopathic Pulmonary Fibrosis and Systemic Autoimmune Rheumatic Disease\u2013Associated Interstitial Lung Disease: Insights From an Italian Multicenter Cohort"],"prefix":"10.3899","volume":"53","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-1716-7432","authenticated-orcid":false,"given":"Marco","family":"Fornaro","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Donato","family":"Lacedonia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Cavagna","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Paolo","family":"Air\u00f2","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1294-6421","authenticated-orcid":false,"given":"Marco","family":"Sebastiani","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Gianluca","family":"Sambataro","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Fabio","family":"Cacciapaglia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Angelica","family":"Napoletano","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cosimo Carlo","family":"De Pace","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-1202-8567","authenticated-orcid":false,"given":"Pasquale","family":"Tondo","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Giulia","family":"Scioscia","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eleonora","family":"Pedretti","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Grazia","family":"Lazzaroni","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Carlo","family":"Vancheri","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andreina","family":"Manfredi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lorenzo","family":"Bianchessi","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rocco","family":"Capece","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Amato A. Stabile","family":"Ianora","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Massimo","family":"Giotta","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0474-5344","authenticated-orcid":false,"given":"Florenzo","family":"Iannone","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"2262","published-online":{"date-parts":[[2026,2,1]]},"reference":[{"key":"2026040105300597000_53.4.450.1","doi-asserted-by":"crossref","first-page":"16689","DOI":"10.3390\/ijerph192416689","article-title":"Prognosis and survival in idiopathic pulmonary fibrosis in the era of antifibrotic therapy in Italy: evidence from a longitudinal population study based on healthcare utilization databases","volume":"19","author":"Iommi","year":"2022","journal-title":"Int J Environ Res Public Health"},{"key":"2026040105300597000_53.4.450.2","doi-asserted-by":"publisher","DOI":"10.1183\/09031936.00106011"},{"key":"2026040105300597000_53.4.450.3","first-page":"288","article-title":"Clinical, histologic and prognostic features of clinically amyopathic dermatomyositis","volume":"42","author":"Fornaro","year":"2024","journal-title":"Clin Exp Rheumatol"},{"key":"2026040105300597000_53.4.450.4","doi-asserted-by":"crossref","first-page":"304","DOI":"10.1016\/S2213-2600(19)30480-1","article-title":"Systemic sclerosis-associated interstitial lung disease","volume":"8","author":"Perelas","year":"2020","journal-title":"Lancet Respir Med"},{"key":"2026040105300597000_53.4.450.5","doi-asserted-by":"crossref","first-page":"156","DOI":"10.4103\/atm.atm_632_20","article-title":"Clinical characteristics and outcomes in patients with primary Sjogren\u2019s syndrome-associated interstitial lung disease","volume":"16","author":"Alhamad","year":"2021","journal-title":"Ann Thorac Med"},{"key":"2026040105300597000_53.4.450.6","doi-asserted-by":"crossref","first-page":"4453","DOI":"10.3390\/ijms25084453","article-title":"A review of antisynthetase syndrome-associated interstitial lung disease","volume":"25","author":"Patel","year":"2024","journal-title":"Int J Mol Sci"},{"key":"2026040105300597000_53.4.450.7","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2011.08.015"},{"key":"2026040105300597000_53.4.450.8","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.200607-912OC"},{"key":"2026040105300597000_53.4.450.9","first-page":"386","article-title":"Clinical characteristics and survival in idiopathic pulmonary fibrosis and connective tissue disease-associated usual interstitial pneumonia","volume":"7","author":"Alhamad","year":"2015","journal-title":"J Thorac Dis"},{"key":"2026040105300597000_53.4.450.10","doi-asserted-by":"crossref","first-page":"99","DOI":"10.1007\/s00296-023-05505-0","article-title":"Survival differences in rheumatoid arthritis interstitial lung disease and idiopathic pulmonary fibrosis may be explained by delays in presentation: results from multivariate analysis in a monocentric UK study","volume":"44","author":"Dubey","year":"2024","journal-title":"Rheumatol Int"},{"key":"2026040105300597000_53.4.450.11","doi-asserted-by":"crossref","first-page":"6508","DOI":"10.3390\/jcm13216508","article-title":"Pulmonary progressive fibrosis in rheumatoid arthritis and primary Sjogren syndrome: similarities and differences","volume":"13","author":"Manfredi","year":"2024","journal-title":"J Clin Med"},{"key":"2026040105300597000_53.4.450.12","doi-asserted-by":"crossref","first-page":"2271","DOI":"10.1002\/art.40320","article-title":"2017 European League Against Rheumatism\/American College of Rheumatology classification criteria for adult and juvenile idiopathic inflammatory myopathies and their major subgroups","volume":"69","author":"Lundberg","year":"2017","journal-title":"Arthritis Rheumatol"},{"key":"2026040105300597000_53.4.450.13","doi-asserted-by":"crossref","first-page":"870","DOI":"10.1016\/j.semarthrit.2017.10.006","article-title":"2013 ACR\/EULAR systemic sclerosis classification criteria in patients with associated pulmonary arterial hypertension","volume":"47","author":"Joven","year":"2018","journal-title":"Semin Arthritis Rheum"},{"key":"2026040105300597000_53.4.450.14","doi-asserted-by":"crossref","first-page":"35","DOI":"10.1002\/art.39859","article-title":"2016 American College of Rheumatology\/European League Against Rheumatism classification criteria for primary Sjogren\u2019s syndrome: a consensus and data-driven methodology involving three international patient cohorts","volume":"69","author":"Shiboski","year":"2016","journal-title":"Arthritis Rheumatol"},{"key":"2026040105300597000_53.4.450.15","doi-asserted-by":"crossref","first-page":"2013","DOI":"10.3390\/jcm8112013","article-title":"Influence of antisynthetase antibodies specificities on antisynthetase syndrome clinical spectrum time course","volume":"8","author":"Cavagna","year":"2019","journal-title":"J Clin Med"},{"key":"2026040105300597000_53.4.450.16","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201807-1255ST"},{"key":"2026040105300597000_53.4.450.17","doi-asserted-by":"publisher","DOI":"10.1002\/art.21322"},{"key":"2026040105300597000_53.4.450.18","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1186\/s12931-014-0154-6","article-title":"Predictors of diagnosis and survival in idiopathic pulmonary fibrosis and connective tissue disease-related usual interstitial pneumonia","volume":"15","author":"Moua","year":"2014","journal-title":"Respir Res"},{"key":"2026040105300597000_53.4.450.19","first-page":"384","article-title":"Myositis-associated usual interstitial pneumonia has a better survival than idiopathic pulmonary fibrosis","volume":"56","author":"Aggarwal","year":"2017","journal-title":"Rheumatology"},{"key":"2026040105300597000_53.4.450.20","doi-asserted-by":"publisher","DOI":"10.1016\/j.chest.2022.11.035"}],"container-title":["The Journal of Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/syndication.highwire.org\/content\/doi\/10.3899\/jrheum.2025-0610","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,4,1]],"date-time":"2026-04-01T12:30:51Z","timestamp":1775046651000},"score":1,"resource":{"primary":{"URL":"http:\/\/www.jrheum.org\/lookup\/doi\/10.3899\/jrheum.2025-0610"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,2,1]]},"references-count":20,"journal-issue":{"issue":"4","published-online":{"date-parts":[[2026,4,1]]},"published-print":{"date-parts":[[2026,4]]}},"alternative-id":["10.3899\/jrheum.2025-0610"],"URL":"https:\/\/doi.org\/10.3899\/jrheum.2025-0610","relation":{},"ISSN":["0315-162X","1499-2752"],"issn-type":[{"value":"0315-162X","type":"print"},{"value":"1499-2752","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,2,1]]}}}