{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,18]],"date-time":"2026-05-18T23:32:06Z","timestamp":1779147126568,"version":"3.51.4"},"reference-count":36,"publisher":"Wiley","issue":"11","license":[{"start":{"date-parts":[[2022,8,24]],"date-time":"2022-08-24T00:00:00Z","timestamp":1661299200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Int J of Rheum Dis"],"published-print":{"date-parts":[[2022,11]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Aim<\/jats:title><jats:p>Adult\u2010onset Still\u2019s disease (ASD) is a rare, potentially life\u2010threatening autoinflammatory condition. As reported prevalence shows regional variation and long\u2010term outcome data are scarce, we investigated epidemiology and long\u2010term health outcomes of ASD in Western Australia (WA).<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>Population\u2010based cohort study using longitudinally linked administrative health data from all WA hospitals between 1999 and 2013 for ASD patients (ICD\u201010\u2010AM\u2009M06.1) and controls matched for age, gender, and index year. Rate ratios and odds ratios (RR\/OR) with 95% confidence intervals (CI) compared ASD patients with controls.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>The average ASD incidence (n\u00a0=\u00a052) was 0.22\/100\u2009000 with 2.4\/100\u2009000 point\u2010prevalence as of December 31, 2013. ASD patients (median age 41.5\u2009years, 59.6% female) had higher odds of previous liver disease (OR 2.67, 95% CI 1.31\u20105.45), fever (OR 54.10, 95% CI 6.60\u2010433.0), rash (OR 15.70, 95% CI 4.08\u201060.80), and serious infections (OR 4.36, 95% CI 2.11\u201022.80) than controls. Despite biological disease\u2010modifying antirheumatic drugs in 27% of patients, ASD patients had higher odds for joint replacement (n\u00a0=\u00a07, 13.5%) (OR 45.5, 95% CI 4.57\u201093.70), osteoporosis (OR 31.3, 95% CI 3.43\u201097), and serious infections (RR 5.68; 95% CI 6.61\u20108.74) during follow up. However, crude mortality (11.5% vs 7.5%; <jats:italic>P<\/jats:italic>\u00a0=\u00a00.34), survival at 1 and 5\u2009years (<jats:italic>P<\/jats:italic>=\u20090.78), and last modified Charlson Comorbidity score (median 2 vs 2) were similar between groups.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>The epidemiology and demographics of ASD in Western Australia fall within the internationally reported range. ASD patients present increased rates of liver disease, rash, and serious infections before disease onset. Mortality following ASD was not increased for 5\u2009years despite high rates of chronic arthritis requiring joint replacement, serious infections, and osteoporosis.<\/jats:p><\/jats:sec>","DOI":"10.1111\/1756-185x.14424","type":"journal-article","created":{"date-parts":[[2022,8,25]],"date-time":"2022-08-25T06:48:39Z","timestamp":1661410119000},"page":"1306-1314","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":5,"title":["Adult\u2010onset Still's disease in Western Australia: Epidemiology, comorbidity and long\u2010term outcome"],"prefix":"10.1111","volume":"25","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-2833-7997","authenticated-orcid":false,"given":"Johannes","family":"Nossent","sequence":"first","affiliation":[{"name":"Department of Rheumatology Sir Charles Gairdner Hospital  Perth Western Australia Australia"},{"name":"Rheumatology Group, School of Medicine University Western Australia  Perth Western Australia 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Health  Perth Western Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0535-963X","authenticated-orcid":false,"given":"Charles A.","family":"Inderjeeth","sequence":"additional","affiliation":[{"name":"Department of Rheumatology Sir Charles Gairdner Hospital  Perth Western Australia Australia"},{"name":"Rheumatology Group, School of Medicine University Western Australia  Perth Western Australia Australia"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"311","published-online":{"date-parts":[[2022,8,24]]},"reference":[{"key":"e_1_2_9_2_1","first-page":"1","article-title":"A form of chronic joint disease in children","volume":"80","author":"Still GF","year":"1897","journal-title":"Med\u2010Chir 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