{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,6]],"date-time":"2026-10-06T18:50:08Z","timestamp":1791312608038,"version":"4.3.1"},"reference-count":22,"publisher":"Wiley","issue":"12","license":[{"start":{"date-parts":[[2016,10,1]],"date-time":"2016-10-01T00:00:00Z","timestamp":1475280000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"funder":[{"DOI":"10.13039\/100006108","name":"National Center for Advancing Translational Sciences","doi-asserted-by":"publisher","award":["UL1 TR000135"],"award-info":[{"award-number":["UL1 TR000135"]}],"id":[{"id":"10.13039\/100006108","id-type":"DOI","asserted-by":"publisher"}]},{"DOI":"10.13039\/100000049","name":"National Institute on Aging","doi-asserted-by":"publisher","award":["R0 1AG034676"],"award-info":[{"award-number":["R0 1AG034676"]}],"id":[{"id":"10.13039\/100000049","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":["Arthritis Care &amp; Research"],"published-print":{"date-parts":[[2016,12]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>To characterize the epidemiology of mixed connective tissue disease (MCTD) from 1983 to 2014.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>An inception cohort of patients with incident MCTD in 1985\u20132014 in Olmsted County, Minnesota was identified based on comprehensive individual medical record review. Diagnosis of MCTD required fulfillment of at least 1 of the 4 widely accepted diagnostic criteria without fulfillment of classification criteria for other connective tissue diseases. Data were collected on demographic characteristics, clinical presentation, laboratory investigations, and mortality.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 50 incident cases of MCTD were identified (mean age 48.1 years and 84% were female). The annual incidence of MCTD was 1.9 per 100,000 population. Raynaud's phenomenon was the most common initial symptoms (50%), followed by arthralgia (30%) and swollen hands (16%). The diagnosis was frequently delayed with the median time from first symptom to fulfillment of criteria of 3.6 years. At fulfillment of criteria, arthralgia was the most prevalent manifestation (86%), followed by Raynaud's phenomenon (80%), swollen hands (64%), leukopenia\/lymphopenia (44%), and heartburn (38%). Evolution to other connective tissue occurred infrequently with a 10\u2010year rate of evolution of 8.5% and 6.3% for systemic lupus erythematosus and systemic sclerosis, respectively. The overall mortality was not different from the general population with a standardized mortality ratio of 1.1 (95% confidence interval 0.4\u20132.6).<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>This study was the first population\u2010based study of MCTD to provide a complete picture of epidemiology and clinical characteristics of MCTD. MCTD occurred in about 2 persons per 100,000 per year. Evolution to other connective diseases occurred infrequently and the mortality was not affected.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.22872","type":"journal-article","created":{"date-parts":[[2016,3,6]],"date-time":"2016-03-06T02:39:30Z","timestamp":1457231970000},"page":"1843-1848","source":"Crossref","is-referenced-by-count":102,"title":["Epidemiology of Mixed Connective Tissue Disease, 1985\u20132014: A Population\u2010Based Study"],"prefix":"10.1002","volume":"68","author":[{"given":"Patompong","family":"Ungprasert","sequence":"first","affiliation":[{"name":"Mayo Clinic Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Cynthia S.","family":"Crowson","sequence":"additional","affiliation":[{"name":"Mayo Clinic Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Vaidehi R.","family":"Chowdhary","sequence":"additional","affiliation":[{"name":"Mayo Clinic Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Floranne C.","family":"Ernste","sequence":"additional","affiliation":[{"name":"Mayo Clinic Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kevin G.","family":"Moder","sequence":"additional","affiliation":[{"name":"Mayo Clinic Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Eric L.","family":"Matteson","sequence":"additional","affiliation":[{"name":"Mayo Clinic Rochester Minnesota"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2016,10]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rdc.2005.04.007"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1016\/0002-9343(72)90064-2"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1046\/j.1365-2796.1996.14843000.x"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2010.143792"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780380216"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/1529-0131(199905)42:5<899::AID-ANR8>3.0.CO;2-L"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.mayocp.2011.11.009"},{"key":"e_1_2_6_9_1","doi-asserted-by":"publisher","DOI":"10.1093\/aje\/kwq482"},{"key":"e_1_2_6_10_1","first-page":"23","volume-title":"Mixed connective tissue disease and anti\u2010nuclear antibodies","author":"Sharp GC.","year":"1987"},{"key":"e_1_2_6_11_1","first-page":"33","volume-title":"Mixed connective tissue disease and anti\u2010nuclear antibodies","author":"Alarc\u00f3n\u2010Segovia D","year":"1987"},{"key":"e_1_2_6_12_1","first-page":"41","volume-title":"Mixed connective tissue disease and anti\u2010nuclear antibodies","author":"Kasukawa R","year":"1987"},{"key":"e_1_2_6_13_1","first-page":"545","volume-title":"Les maladies syst\u00e9miques","author":"Kahn MF","year":"1991"},{"key":"e_1_2_6_14_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.34473"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2013-204424"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197502132920706"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJM197502202920807"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1136\/ard.2010.138461"},{"key":"e_1_2_6_19_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.semarthrit.2011.07.010"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1136\/annrheumdis-2011-201253"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.rmed.2008.12.018"},{"key":"e_1_2_6_22_1","doi-asserted-by":"publisher","DOI":"10.1097\/00005792-198007000-00001"},{"key":"e_1_2_6_23_1","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.121272"}],"container-title":["Arthritis Care &amp; 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