{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,1]],"date-time":"2026-10-01T06:56:40Z","timestamp":1790837800092,"version":"4.1.0"},"reference-count":42,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:00:00Z","timestamp":1790726400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc\/4.0\/"},{"start":{"date-parts":[[2026,9,30]],"date-time":"2026-09-30T00:00:00Z","timestamp":1790726400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"abstract":"<jats:sec>\n                    <jats:title>Objective<\/jats:title>\n                    <jats:p>We evaluated baseline characteristics, treatment patterns, and outcomes in patients with systemic autoimmune rheumatic disease\u2013associated progressive pulmonary fibrosis (SARD\u2010PPF) and evaluated whether outcomes differed by SARD subtype.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>The ILD\u2010PRO Registry is a prospective multicenter US registry of patients with PPF. Eligible participants had an interstitial lung disease (ILD) other than idiopathic pulmonary fibrosis with reticulation and traction bronchiectasis on high\u2010resolution computed tomography and\/or lung biopsy and met the criteria for PPF within the prior 24 months. Among patients with SARD\u2010PPF, we described baseline characteristics and evaluated associations between SARD subtype and clinical outcomes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>Among 585 patients with SARD\u2010PPF, physiologic impairment at enrollment was substantial (median forced vital capacity [FVC] 64.5% predicted; median diffusing capacity of the lungs for carbon monoxide 38.0% predicted): 39.2% used supplemental oxygen, 73.9% were receiving immunomodulatory therapy, and 22.6% were taking nintedanib. By 24 months, 31.3% to 62.1% of patients experienced ILD progression across SARD subtypes, and 9.3% to 37.6% experienced death or lung transplant. Rheumatoid arthritis\u2010PPF showed the highest unadjusted probability of ILD progression; however, no significant subtype\u2010associated differences were observed in analyses adjusted for age, sex, and\/or baseline FVC % predicted.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>In a large prospective multicenter US cohort, SARD\u2010PPF was characterized by advanced physiologic impairment, high treatment burden, and high risk for further progression and death or lung transplant. After accounting for demographic factors and baseline severity, outcomes were broadly similar across SARD diagnoses, supporting phenotype\u2010focused risk stratification and underscoring the need for systematic monitoring and timely optimization of management for SARD\u2010ILD.<\/jats:p>\n                    <jats:p>\n                      <jats:boxed-text content-type=\"graphic\" position=\"anchor\">\n                        <jats:graphic xmlns:xlink=\"http:\/\/www.w3.org\/1999\/xlink\" mimetype=\"image\/png\" position=\"anchor\" specific-use=\"enlarged-web-image\" xlink:href=\"graphic\/art70283-gra-0001-m.png\">\n                          <jats:alt-text>image<\/jats:alt-text>\n                        <\/jats:graphic>\n                      <\/jats:boxed-text>\n                    <\/jats:p>\n                  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