{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,9]],"date-time":"2026-05-09T07:09:50Z","timestamp":1778310590813,"version":"3.51.4"},"reference-count":11,"publisher":"SAGE Publications","license":[{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,5,1]],"date-time":"2026-05-01T00:00:00Z","timestamp":1777593600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/journals.sagepub.com\/page\/policies\/text-and-data-mining-license"}],"content-domain":{"domain":["journals.sagepub.com"],"crossmark-restriction":true},"short-container-title":["Ther Adv Respir Dis"],"published-print":{"date-parts":[[2026,5]]},"abstract":"<jats:sec>\n                    <jats:title>Background:<\/jats:title>\n                    <jats:p>Pleuroparenchymal fibroelastosis (PPFE) is a rare interstitial lung disease (ILD) associated with systemic autoimmune rheumatic diseases (SARDs). Its clinical characteristics and outcomes in SARDs remain poorly defined.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Objectives:<\/jats:title>\n                    <jats:p>To describe demographics, imaging patterns, and outcomes in SARD-associated PPFE.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods:<\/jats:title>\n                    <jats:p>Patients diagnosed with PPFE and SARDs at Mayo Clinic (2000-2024) were included. PPFE diagnosis was confirmed by high-resolution computed tomography, and SARD diagnoses were established by rheumatologists.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results:<\/jats:title>\n                    <jats:p>Fifteen of 62 patients with PPFE had SARDs with majority having systemic sclerosis (SSc)(n=\u20097, 46%). Median age at PPFE diagnosis was 57.2 years; 80% were female; 40% were ever-smokers; 40% required supplemental oxygen; 5 had pulmonary hypertension. Coexisting ILD patterns included UIP (26.7%) and NSIP (20%). Coexisting ILD patterns included usual interstitial pneumonia (26.7%) and nonspecific interstitial pneumonia (20%). Three patients died, including two with SSc.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion:<\/jats:title>\n                    <jats:p>Systemic sclerosis was the most frequently observed SARD among patients with PPFE, and may coexist with other ILD patterns; recognition may inform risk stratification, multidisciplinary evaluation, and longitudinal follow-up.<\/jats:p>\n                  <\/jats:sec>","DOI":"10.1177\/17534666261445202","type":"journal-article","created":{"date-parts":[[2026,5,6]],"date-time":"2026-05-06T11:18:59Z","timestamp":1778066339000},"update-policy":"https:\/\/doi.org\/10.1177\/sage-journals-update-policy","source":"Crossref","is-referenced-by-count":0,"title":["Pleuroparenchymal fibroelastosis from a rheumatologic perspective"],"prefix":"10.1177","volume":"20","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-9117-7535","authenticated-orcid":false,"given":"Maximiliano","family":"Diaz Menindez","sequence":"first","affiliation":[{"name":"Department of Medicine, Mayo Clinic Arizona, Phoenix, AZ, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Alejandro","family":"Diaz Arumir Vergara","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Sleep Medicine, Mayo Clinic Arizona, Phoenix, AZ, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vivian","family":"Shing","sequence":"additional","affiliation":[{"name":"Mayo Clinic Alix School of Medicine, Phoenix, AZ, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Carolyn","family":"Mead Harvey","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Sleep Medicine, Mayo Clinic Arizona, Phoenix, AZ, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Ana C.","family":"Zamora Martinez","sequence":"additional","affiliation":[{"name":"Division of Pulmonary and Sleep Medicine, Mayo Clinic Arizona, Phoenix, AZ, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Vivek","family":"Nagaraja","sequence":"additional","affiliation":[{"name":"Division of Rheumatology, Mayo Clinic Arizona, 13400 E Shea Boulevard, Scottsdale, AZ 85259, USA"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"179","published-online":{"date-parts":[[2026,5,6]]},"reference":[{"key":"e_1_3_4_2_2","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keaa451"},{"key":"e_1_3_4_3_2","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keaa879"},{"key":"e_1_3_4_4_2","doi-asserted-by":"publisher","DOI":"10.1164\/rccm.201308-1483ST"},{"key":"e_1_3_4_5_2","doi-asserted-by":"publisher","DOI":"10.1183\/13993003.02135-2019"},{"key":"e_1_3_4_6_2","doi-asserted-by":"publisher","DOI":"10.1513\/AnnalsATS.201902-181CME"},{"key":"e_1_3_4_7_2","doi-asserted-by":"publisher","DOI":"10.1186\/s12890-018-0641-5"},{"key":"e_1_3_4_8_2","doi-asserted-by":"publisher","DOI":"10.1186\/s12931-022-02064-z"},{"key":"e_1_3_4_9_2","doi-asserted-by":"publisher","DOI":"10.2174\/1573397118666220818095927"},{"key":"e_1_3_4_10_2","doi-asserted-by":"publisher","DOI":"10.1093\/mrcr\/rxad035"},{"key":"e_1_3_4_11_2","doi-asserted-by":"publisher","DOI":"10.1136\/bcr-2017-223287"},{"key":"e_1_3_4_12_2","doi-asserted-by":"publisher","DOI":"10.7326\/0003-4819-147-8-200710160-00010"}],"container-title":["Therapeutic 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