{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,5]],"date-time":"2026-10-05T16:24:02Z","timestamp":1791217442431,"version":"4.1.0"},"reference-count":36,"publisher":"MDPI AG","issue":"21","license":[{"start":{"date-parts":[[2021,10,21]],"date-time":"2021-10-21T00:00:00Z","timestamp":1634774400000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"name":"Skibsreder Per Henriksen &amp; Hustru\u2019s Fond.","award":["No specific number"],"award-info":[{"award-number":["No specific number"]}]},{"DOI":"10.13039\/100008368","name":"Gigtforeningen","doi-asserted-by":"publisher","award":["R160-A5098"],"award-info":[{"award-number":["R160-A5098"]}],"id":[{"id":"10.13039\/100008368","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["JCM"],"abstract":"<jats:p>Objectives: Interstitial lung disease (ILD) is associated with impaired survival among patients with connective tissue diseases (CTDs), but population-based data on the frequency of ILD and pulmonary hypertension (PH) in different CTD subtypes and the impact on survival are sparse. Methods: We included patients with a first-time ICD-10 diagnosis of systemic sclerosis (SSc), mixed connective tissue disease (MCTD), myositis, systemic lupus erythematosus (SLE), or Sj\u00f6gren\u2019s disease registered in the Danish National Patient Registry between 2000 and 2015. Among these, we identified patients with ILD and PH. Using Kaplan\u2013Meier analysis, we assessed survival for the five subtypes of CTD \u00b1 ILD and compared survival among CTD patients overall \u00b1 ILD with survival in the general population \u00b1 ILD. Results: We identified 11,731 patients with a diagnosis of CTD; 637 (5.4%) had a diagnosis of ILD. The proportion of patients with ILD was higher in SSc (13.4%) and MCTD (9.1%) than in myositis (6.0%), SLE (4.1%), and Sj\u00f6gren (2.8%). Fifty-one percent were diagnosed with ILD in their fifties and sixties. PH was more frequent in SSc (7.5%) and MCTD (4.1%). Five-year survival was 73.3% (66.7\u201380.6) in SSc-ILD, 81.0% (69.0\u201395.1) in MCTD-ILD, 84.7% (77.3\u201392.9) in myositis-ILD, 83.5% (76.2\u201391.5) in SLE-ILD, and 84.7 (78.4\u201391.6) in Sj\u00f6gren-associated ILD. Survival in CTD-ILD overall was impaired for all age groups compared with CTD alone. Age-stratified survival was comparable between CTD-ILD and ILD in the general population. The survival gap between ILD and non-ILD increased with age. Conclusion: Survival was comparable between different CTD-ILD subtypes and comparable to survival in non-CTD-ILD.<\/jats:p>","DOI":"10.3390\/jcm10214830","type":"journal-article","created":{"date-parts":[[2021,10,22]],"date-time":"2021-10-22T00:22:02Z","timestamp":1634862122000},"page":"4830","update-policy":"https:\/\/doi.org\/10.3390\/mdpi_crossmark_policy","source":"Crossref","is-referenced-by-count":46,"title":["Interstitial Lung Disease in Connective Tissue Diseases: Survival Patterns in a Population-Based Cohort"],"prefix":"10.3390","volume":"10","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-6353-8671","authenticated-orcid":false,"given":"Charlotte","family":"Hyldgaard","sequence":"first","affiliation":[{"name":"Diagnostic Centre, Silkeborg Regional Hospital, University Clinic for Innovative Patient Pathways, Aarhus University, 8600 Silkeborg, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4238-6963","authenticated-orcid":false,"given":"Elisabeth","family":"Bendstrup","sequence":"additional","affiliation":[{"name":"Centre for Rare Lung Diseases, Department of Respiratory Diseases and Allergy, Aarhus University Hospital, 8200 Aarhus, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alma Becic","family":"Pedersen","sequence":"additional","affiliation":[{"name":"Department of Clinical Epidemiology, Aarhus University Hospital, 8200 Aarhus, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Lars","family":"Pedersen","sequence":"additional","affiliation":[{"name":"Department of Clinical Epidemiology, Aarhus University Hospital, 8200 Aarhus, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0426-4962","authenticated-orcid":false,"given":"Torkell","family":"Ellingsen","sequence":"additional","affiliation":[{"name":"Diagnostic Centre, Silkeborg Regional Hospital, University Clinic for Innovative Patient Pathways, Aarhus University, 8600 Silkeborg, Denmark"},{"name":"Rheumatology Research Unit, Odense University Hospital, University of Southern Denmark, 5000 Odense, Denmark"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"1968","published-online":{"date-parts":[[2021,10,21]]},"reference":[{"key":"ref_1","doi-asserted-by":"crossref","first-page":"h6819","DOI":"10.1136\/bmj.h6819","article-title":"Management of interstitial lung disease associated with connective tissue disease","volume":"352","author":"Mathai","year":"2016","journal-title":"BMJ"},{"key":"ref_2","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 Rheumatol."},{"key":"ref_3","doi-asserted-by":"crossref","first-page":"1623","DOI":"10.1093\/rheumatology\/key139","article-title":"The Scleroderma Patient-Centered Intervention Network Cohort: Baseline clinical features and comparison with other large scleroderma cohorts","volume":"57","author":"Dougherty","year":"2018","journal-title":"Rheumatology"},{"key":"ref_4","doi-asserted-by":"crossref","first-page":"17","DOI":"10.1186\/s13075-018-1517-z","article-title":"Systemic sclerosis associated interstitial lung disease\u2014Individualized immunosuppressive therapy and course of lung function: Results of the EUSTAR group","volume":"20","author":"Adler","year":"2018","journal-title":"Arthritis Res."},{"key":"ref_5","doi-asserted-by":"crossref","first-page":"1258","DOI":"10.1164\/rccm.201903-0486OC","article-title":"Tracking Impact of Interstitial Lung Disease in Systemic Sclerosis in a Complete Nationwide Cohort","volume":"200","author":"Fretheim","year":"2019","journal-title":"Am. 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