{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T00:37:44Z","timestamp":1792802264025,"version":"4.5.0"},"reference-count":27,"publisher":"Springer Science and Business Media LLC","license":[{"start":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T00:00:00Z","timestamp":1792713600000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T00:00:00Z","timestamp":1792713600000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"GSK","award":["223907"],"award-info":[{"award-number":["223907"]}]},{"name":"GSK","award":["224023"],"award-info":[{"award-number":["224023"]}]}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Clin Rheumatol"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title>Objectives<\/jats:title>\n                    <jats:p>Interstitial lung disease (ILD) is a pulmonary manifestation of connective tissue diseases (CTDs). Limited data exist describing the epidemiology of CTD-ILD; therefore, this study aimed to estimate the incidence and prevalence of CTD-ILD in the US, overall and by CTD-ILD subtypes.<\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Methods<\/jats:title>\n                    <jats:p>\n                      Patients with CTD-ILD and rheumatoid arthritis (RA), systemic sclerosis (SSc), systemic lupus erythematosus (SLE), idiopathic inflammatory myopathy (IIM), mixed CTD (MCTD), and Sj\u00f6gren\u2019s disease (SjD) were identified from Optum\n                      <jats:sup>\u00ae<\/jats:sup>\n                      de-identified Electronic Health Record data set (Optum\n                      <jats:sup>\u00ae<\/jats:sup>\n                      EHR) and MarketScan\n                      <jats:sup>\u00ae<\/jats:sup>\n                      Commercial and Medicare US databases between 2019 and 2024.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Results<\/jats:title>\n                    <jats:p>\n                      ILD was present in 4.3% (n\u2009=\u200921,366\/496,690; Optum\n                      <jats:sup>\u00ae<\/jats:sup>\n                      EHR) and 3.2% (n\u2009=\u20097521\/234,468; MarketScan) of identified patients with CTD. This proportion was highest for those with SSc (Optum\n                      <jats:sup>\u00ae<\/jats:sup>\n                      EHR: 20.3%; MarketScan: 21.1%).\u00a0Most patients with CTD-ILD were female in both Optum\n                      <jats:sup>\u00ae<\/jats:sup>\n                      EHR and MarketScan, with median ages 64 and 56 years, respectively; race was available only in Optum\n                      <jats:sup>\u00ae<\/jats:sup>\n                      EHR, where 72.1% were categorized as Caucasian. Age- and sex-adjusted incidence rates were 7.6 and 10.0 per 100,000 person-years, with adjusted prevalence rates 46.7 and 43.4 per 100,000 persons. Rates varied by CTD subtype and were highest for RA-ILD and lowest for MCTD-ILD across databases. Crude rates of CTD-ILD regardless of subtype were higher among female patients, patients aged\u2009\u2265\u200965 years, and among patients of Black African ancestry.\n                    <\/jats:p>\n                  <\/jats:sec>\n                  <jats:sec>\n                    <jats:title>Conclusion<\/jats:title>\n                    <jats:p>Consistent age- and sex-adjusted CTD-ILD incidence and prevalence estimates between two large, demographically distinct US databases provide contemporary population-level estimates of CTD-ILD frequency and distribution that can inform disease screening and patient care.<\/jats:p>\n                    <jats:p>\n                      <jats:table-wrap>\n                        <jats:table>\n                          <jats:tbody>\n                            <jats:tr>\n                              <jats:td align=\"left\" colspan=\"3\">\n                                <jats:bold>Key points<\/jats:bold>\n                                \u2022\u00a0\n                                <jats:italic>US CTD-ILD incidence estimated at 7.6 and 10.0\/100,000 person-years and prevalence estimated at 46.7 and 43.4\/100,000 persons.<\/jats:italic>\n                                \u2022\u00a0\n                                <jats:italic>Incidence and prevalence rates were highest in female patients, patients aged \u2265 65 years, and patients of Black African ancestry.<\/jats:italic>\n                                \u2022\u00a0\n                                <jats:italic>Incidence and prevalence rates varied between CTD subtypes and were highest for RA-ILD and lowest for MCTD-ILD.<\/jats:italic>\n                              <\/jats:td>\n                            <\/jats:tr>\n                          <\/jats:tbody>\n                        <\/jats:table>\n                      <\/jats:table-wrap>\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1007\/s10067-026-08480-6","type":"journal-article","created":{"date-parts":[[2026,10,23]],"date-time":"2026-10-23T07:03:13Z","timestamp":1792738993000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Incidence and prevalence of connective tissue disease (CTD)-associated interstitial lung disease in the United States"],"prefix":"10.1007","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-8330-7996","authenticated-orcid":false,"given":"Diana","family":"Martins","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8977-3189","authenticated-orcid":false,"given":"George","family":"Mu","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-3437-2526","authenticated-orcid":false,"given":"Elaine","family":"Irving","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0005-4918-6939","authenticated-orcid":false,"given":"Sally","family":"Wetten","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-6393-6031","authenticated-orcid":false,"given":"Roger A.","family":"Levy","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5177-1759","authenticated-orcid":false,"given":"Keele","family":"Wurst","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2026,10,23]]},"reference":[{"key":"8480_CR1","doi-asserted-by":"publisher","first-page":"e20230132","DOI":"10.36416\/1806-3756\/e20230132","volume":"50","author":"KM Storrer","year":"2024","unstructured":"Storrer KM, Muller CS, Pessoa MCA, Pereira CAC (2024) Connective tissue disease-associated interstitial lung disease. J Bras Pneumol 50:e20230132. https:\/\/doi.org\/10.36416\/1806-3756\/e20230132","journal-title":"J Bras Pneumol"},{"key":"8480_CR2","doi-asserted-by":"publisher","first-page":"180076","DOI":"10.1183\/16000617.0076-2018","volume":"27","author":"V Cottin","year":"2018","unstructured":"Cottin V, Hirani NA, Hotchkin DL et al (2018) Presentation, diagnosis and clinical course of the spectrum of progressive-fibrosing interstitial lung diseases. Eur Respir Rev 27:180076. https:\/\/doi.org\/10.1183\/16000617.0076-2018","journal-title":"Eur Respir Rev"},{"key":"8480_CR3","doi-asserted-by":"publisher","first-page":"769","DOI":"10.1016\/S0140-6736(22)01052-2","volume":"400","author":"M Wijsenbeek","year":"2022","unstructured":"Wijsenbeek M, Suzuki A, Maher TM (2022) Interstitial lung diseases. Lancet 400:769\u2013786. https:\/\/doi.org\/10.1016\/S0140-6736(22)01052-2","journal-title":"Lancet"},{"key":"8480_CR4","unstructured":"Kamrani P, Marston G, Arbor TC, Jan A (2025) Anatomy, connective tissue. StatPearls. Treasure Island (FL)"},{"key":"8480_CR5","doi-asserted-by":"publisher","first-page":"100419","DOI":"10.1016\/j.ejro.2022.100419","volume":"9","author":"H Yoo","year":"2022","unstructured":"Yoo H, Hino T, Hwang J et al (2022) Connective tissue disease-related interstitial lung disease (CTD-ILD) and interstitial lung abnormality (ILA): evolving concept of CT findings, pathology and management. Eur J Radiol Open 9:100419. https:\/\/doi.org\/10.1016\/j.ejro.2022.100419","journal-title":"Eur J Radiol Open"},{"key":"8480_CR6","doi-asserted-by":"publisher","first-page":"e0327462","DOI":"10.1371\/journal.pone.0327462","volume":"20","author":"LL O'Hare","year":"2025","unstructured":"O\u2019Hare LL, Iyer AS, Lindell KO et al (2025) Factors affecting quality of life in connective tissue disease-related interstitial lung disease. PLoS ONE 20:e0327462. https:\/\/doi.org\/10.1371\/journal.pone.0327462","journal-title":"PLoS ONE"},{"key":"8480_CR7","doi-asserted-by":"publisher","first-page":"4830","DOI":"10.3390\/jcm10214830","volume":"10","author":"C Hyldgaard","year":"2021","unstructured":"Hyldgaard C, Bendstrup E, Pedersen AB, Pedersen L, Ellingsen T (2021) Interstitial lung disease in connective tissue diseases: survival patterns in a population-based cohort. J Clin Med 10:4830. https:\/\/doi.org\/10.3390\/jcm10214830","journal-title":"J Clin Med"},{"key":"8480_CR8","doi-asserted-by":"publisher","unstructured":"Vaezi A, Ashby T, Balavenkataraman A, Sweiss N, Mirsaeidi M (2025) Contribution of interstitial lung disease to mortality in patients with connective tissue diseases. Chest Pulmonary 3. https:\/\/doi.org\/10.1016\/j.chpulm.2025.100157","DOI":"10.1016\/j.chpulm.2025.100157"},{"key":"8480_CR9","doi-asserted-by":"publisher","first-page":"735","DOI":"10.1007\/s00408-020-00383-w","volume":"198","author":"N Jeganathan","year":"2020","unstructured":"Jeganathan N, Sathananthan M (2020) Connective tissue disease-related interstitial lung disease: prevalence, patterns, predictors, prognosis, and treatment. Lung 198:735\u2013759. https:\/\/doi.org\/10.1007\/s00408-020-00383-w","journal-title":"Lung"},{"key":"8480_CR10","doi-asserted-by":"publisher","first-page":"689","DOI":"10.1016\/S0140-6736(12)61079-4","volume":"380","author":"A Fischer","year":"2012","unstructured":"Fischer A, du Bois R (2012) Interstitial lung disease in connective tissue disorders. Lancet 380:689\u2013698. https:\/\/doi.org\/10.1016\/S0140-6736(12)61079-4","journal-title":"Lancet"},{"key":"8480_CR11","doi-asserted-by":"publisher","first-page":"1182","DOI":"10.1002\/art.42861","volume":"76","author":"SR Johnson","year":"2024","unstructured":"Johnson SR, Bernstein EJ, Bolster MB et al (2024) 2023 American College of Rheumatology (ACR)\/American College of Chest Physicians (CHEST) guideline for the treatment of interstitial lung disease in people with systemic autoimmune rheumatic diseases. Arthritis Rheumatol 76:1182\u20131200. https:\/\/doi.org\/10.1002\/art.42861","journal-title":"Arthritis Rheumatol"},{"key":"8480_CR12","doi-asserted-by":"publisher","first-page":"220210","DOI":"10.1183\/16000617.0210-2022","volume":"32","author":"GM Joy","year":"2023","unstructured":"Joy GM, Arbiv OA, Wong CK et al (2023) Prevalence, imaging patterns and risk factors of interstitial lung disease in connective tissue disease: a systematic review and meta-analysis. Eur Respir Rev 32:220210. https:\/\/doi.org\/10.1183\/16000617.0210-2022","journal-title":"Eur Respir Rev"},{"key":"8480_CR13","doi-asserted-by":"publisher","first-page":"1077","DOI":"10.1513\/AnnalsATS.201911-822OC","volume":"17","author":"M Algamdi","year":"2020","unstructured":"Algamdi M, Sadatsafavi M, Fisher JH et al (2020) Costs of workplace productivity loss in patients with connective tissue disease-associated interstitial lung disease. Ann Am Thorac Soc 17:1077\u20131084. https:\/\/doi.org\/10.1513\/AnnalsATS.201911-822OC","journal-title":"Ann Am Thorac Soc"},{"key":"8480_CR14","doi-asserted-by":"publisher","first-page":"360","DOI":"10.3899\/jrheum.171315","volume":"46","author":"K Raimundo","year":"2019","unstructured":"Raimundo K, Solomon JJ, Olson AL et al (2019) Rheumatoid arthritis-interstitial lung disease in the United States: prevalence, incidence, and healthcare costs and mortality. J Rheumatol 46:360\u2013369. https:\/\/doi.org\/10.3899\/jrheum.171315","journal-title":"J Rheumatol"},{"key":"8480_CR15","unstructured":"Genentech (2021) Genentech\u2019s Actemra Becomes the First Biologic Therapy Approved by the FDA for Slowing the Rate of Decline in Pulmonary Function in Adults With Systemic Sclerosis-Associated Interstitial Lung Disease, a Rare, Debilitating Condition. https:\/\/www.gene.com\/media\/press-releases\/14897\/2021-03-04\/genentechs-actemra-becomes-the-first-bio. Accessed Oct 2026"},{"key":"8480_CR16","doi-asserted-by":"publisher","first-page":"963","DOI":"10.1016\/s2213-2600(20)30318-0","volume":"8","author":"D Khanna","year":"2020","unstructured":"Khanna D, Lin CJF, Furst DE et al (2020) Tocilizumab in systemic sclerosis: a randomised, double-blind, placebo-controlled, phase 3 trial. Lancet Respir Med 8:963\u2013974. https:\/\/doi.org\/10.1016\/s2213-2600(20)30318-0","journal-title":"Lancet Respir Med"},{"key":"8480_CR17","doi-asserted-by":"publisher","first-page":"674","DOI":"10.1164\/rccm.202103-0714OC","volume":"205","author":"D Khanna","year":"2022","unstructured":"Khanna D, Lin CJF, Furst DE et al (2022) Long-term safety and efficacy of tocilizumab in early systemic sclerosis-interstitial lung disease: open-label extension of a phase 3 randomized controlled trial. Am J Respir Crit Care Med 205:674\u2013684. https:\/\/doi.org\/10.1164\/rccm.202103-0714OC","journal-title":"Am J Respir Crit Care Med"},{"key":"8480_CR18","unstructured":"FDA (2024) Nintedanib Prescribing Information. https:\/\/www.accessdata.fda.gov\/drugsatfda_docs\/label\/2024\/205832s027lbl.pdf. Accessed Oct 2026"},{"key":"8480_CR19","unstructured":"EMA (2025) Nintedanib Summary of Product Characteristics. https:\/\/www.ema.europa.eu\/en\/documents\/product-information\/nintedanib-accord-epar-product-information_en.pdf. Accessed Oct 2026"},{"key":"8480_CR20","doi-asserted-by":"publisher","unstructured":"Antoniou KM, Distler O, Gheorghiu AM, et al (2025) ERS\/EULAR clinical practice guidelines for connective tissue disease-associated interstitial lung disease. Eur Respir J 2500896. https:\/\/doi.org\/10.1183\/13993003.02533-2024","DOI":"10.1183\/13993003.02533-2024"},{"key":"8480_CR21","doi-asserted-by":"publisher","first-page":"1539","DOI":"10.18553\/jmcp.2020.20136","volume":"26","author":"Y Fan","year":"2020","unstructured":"Fan Y, Bender S, Shi W, Zoz D (2020) Incidence and prevalence of systemic sclerosis and systemic sclerosis with interstitial lung disease in the United States. J Manag Care Spec Pharm 26:1539\u20131547. https:\/\/doi.org\/10.18553\/jmcp.2020.20136","journal-title":"J Manag Care Spec Pharm"},{"key":"8480_CR22","doi-asserted-by":"publisher","first-page":"1915","DOI":"10.1093\/rheumatology\/keaa547","volume":"60","author":"Q Li","year":"2021","unstructured":"Li Q, Wallace L, Patnaik P et al (2021) Disease frequency, patient characteristics, comorbidity outcomes and immunosuppressive therapy in systemic sclerosis and systemic sclerosis-associated interstitial lung disease: a US cohort study. Rheumatology (Oxford) 60:1915\u20131925. https:\/\/doi.org\/10.1093\/rheumatology\/keaa547","journal-title":"Rheumatology (Oxford)"},{"key":"8480_CR23","doi-asserted-by":"publisher","first-page":"1392","DOI":"10.1002\/acr.24043","volume":"72","author":"BR England","year":"2020","unstructured":"England BR, Roul P, Mahajan TD et al (2020) Performance of administrative algorithms to identify interstitial lung disease in rheumatoid arthritis. Arthritis Care Res (Hoboken) 72:1392\u20131403. https:\/\/doi.org\/10.1002\/acr.24043","journal-title":"Arthritis Care Res (Hoboken)"},{"key":"8480_CR24","doi-asserted-by":"publisher","first-page":"e001291","DOI":"10.1136\/bmjresp-2022-001291","volume":"10","author":"R Shah Gupta","year":"2023","unstructured":"Shah Gupta R, Koteci A, Morgan A, George PM, Quint JK (2023) Incidence and prevalence of interstitial lung diseases worldwide: a systematic literature review. BMJ Open Respir Res 10:e001291. https:\/\/doi.org\/10.1136\/bmjresp-2022-001291","journal-title":"BMJ Open Respir Res"},{"key":"8480_CR25","doi-asserted-by":"publisher","first-page":"209","DOI":"10.1080\/1744666X.2024.2407536","volume":"21","author":"F Atzeni","year":"2025","unstructured":"Atzeni F, Alciati A, Gozza F, Masala IF, Siragusano C, Pipitone N (2025) Interstitial lung disease in rheumatic diseases: an update of the 2018 review. Expert Rev Clin Immunol 21:209\u2013226. https:\/\/doi.org\/10.1080\/1744666X.2024.2407536","journal-title":"Expert Rev Clin Immunol"},{"key":"8480_CR26","doi-asserted-by":"publisher","first-page":"352","DOI":"10.1016\/j.chest.2024.02.045","volume":"166","author":"SA Guler","year":"2024","unstructured":"Guler SA, Scheschkowski T, Renner A, Kampf L, Gasser M, Maurer B (2024) Interdisciplinary diagnosis and management of patients with interstitial lung disease and connective tissue disease. Chest 166:352\u2013361. https:\/\/doi.org\/10.1016\/j.chest.2024.02.045","journal-title":"Chest"},{"key":"8480_CR27","doi-asserted-by":"publisher","unstructured":"Hannah JR, Gordon PA, Galloway J, et al (2022) Validation of methods to identify people with idiopathic inflammatory myopathies using hospital episode statistics. Rheumatol Adv Pract 6. https:\/\/doi.org\/10.1093\/rap\/rkac102","DOI":"10.1093\/rap\/rkac102"}],"container-title":["Clinical Rheumatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-026-08480-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10067-026-08480-6","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/link.springer.com\/content\/pdf\/10.1007\/s10067-026-08480-6.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T00:02:04Z","timestamp":1792800124000},"score":1,"resource":{"primary":{"URL":"https:\/\/link.springer.com\/article\/10.1007\/s10067-026-08480-6"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2026,10,23]]},"references-count":27,"alternative-id":["8480"],"URL":"https:\/\/doi.org\/10.1007\/s10067-026-08480-6","relation":{},"ISSN":["0770-3198","1434-9949"],"issn-type":[{"value":"0770-3198","type":"print"},{"value":"1434-9949","type":"electronic"}],"subject":[],"published":{"date-parts":[[2026,10,23]]},"assertion":[{"value":"13 March 2026","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"30 September 2026","order":2,"name":"revised","label":"Revised","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"2 October 2026","order":3,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"23 October 2026","order":4,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"This study complied with all applicable laws regarding subject privacy and was conducted in accordance with the Declaration of Helsinki 2008 and its later amendments. No direct subject contact or primary collection of individual human subject data occurred. Study results are in tabular form and presented as aggregate analyses that omit subject identification; therefore, informed consent, ethics committee, or institutional review board approval was not required. Any publications and reports do not include subject identifiers.","order":1,"name":"Ethics","label":"Ethics approval","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"Part of this research was previously presented at the European Alliance of Associations for Rheumatology (EULAR) Congress held in Barcelona, Spain (June 11\u201314, 2025), published in the\n                      Annals of the Rheumatic Diseases\n                      (Martins D, et al.\n                      Ann Rheum Dis\n                      . 2025;84(1):1408\u20139) and the American College of Rheumatology (ACR) 2025 Annual Meeting held in Chicago, USA (October 24\u201329, 2025), and is published in\n                      Arthritis & Rheumatology\n                      (Martins D, et al.\u00a0\n                      Arthritis Rheumatol.\n                      \u00a02025; 77 (suppl 9)).","order":2,"name":"Ethics","label":"Previous presentation","group":{"name":"EthicsHeading","label":"Declarations"}},{"value":"All authors are employees of GSK and hold financial equities in the company.","order":3,"name":"Ethics","label":"Conflict of interest","group":{"name":"EthicsHeading","label":"Declarations"}}]}}