{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,24]],"date-time":"2026-10-24T04:44:37Z","timestamp":1792817077344,"version":"4.5.0"},"reference-count":40,"publisher":"Springer Science and Business Media LLC","issue":"11","license":[{"start":{"date-parts":[[2022,7,29]],"date-time":"2022-07-29T00:00:00Z","timestamp":1659052800000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2022,7,29]],"date-time":"2022-07-29T00:00:00Z","timestamp":1659052800000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"funder":[{"name":"University of Bern"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Clin Rheumatol"],"published-print":{"date-parts":[[2022,11]]},"abstract":"<jats:title>Abstract\n<\/jats:title><jats:sec>\n                <jats:title>Objectives<\/jats:title>\n                <jats:p>As a rare and heterogeneous disease, mixed connective tissue disease (MCTD) represents a challenge. Herein, we aimed to unravel potential pitfalls including correct referral diagnosis, distinction from other connective tissue diseases (CTD) and treatment modalities.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Methods<\/jats:title>\n                <jats:p>We characterised the MCTD cohort at our tertiary referral centre. All patients were evaluated for fulfilment of classification criteria of various CTDs. SLEDAI-2\u00a0K and EUSTAR-AI were used in accordance with previous research to evaluate disease activity and treatment response.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Results<\/jats:title>\n                <jats:p>Out of 85 patients initially referred as MCTD, only one-third (33\/85, 39%) fulfilled the diagnostic MCTD criteria and the other patients had undifferentiated CTD (16\/85, 19%), non-MCTD overlap syndromes (11\/85, 13%) and other rheumatic diseases. In our final cohort of 33 MCTD patients, 16 (48%) also met the diagnostic criteria of systemic sclerosis, 13 (39%) these of systemic lupus erythematosus, 6 (18%) these of rheumatoid arthritis and 3 (9%) these of primary myositis. Management of MCTD required immunomodulating combination therapy in most cases (15\/28, 54%), whereas monotherapy was less frequent (10\/28, 36%), and only a few (3\/28, 11%) remained without immune modulators until the end of the follow-up period. Treatment led to a significant decline in disease activity.<\/jats:p>\n              <\/jats:sec><jats:sec>\n                <jats:title>Conclusions<\/jats:title>\n                <jats:p>Our study showed a high risk for misdiagnosis for patients with MCTD. As a multi-organ disease, MCTD required prolonged immunomodulating therapy to achieve remission. The establishment of an international registry with longitudinal data from observational multi-centre cohorts might represent a first step to address the many unmet needs of MCTD.<\/jats:p>\n              <jats:table-wrap><jats:table><jats:tbody>\n                    <jats:tr>\n                      <jats:td colspan=\"2\"><jats:bold>Key Points<\/jats:bold><jats:italic>\u2022 This cohort study aimed to identify challenges in the highly complex management of MCTD.<\/jats:italic><jats:italic>\u2022 Clinical presentation of MCTD significantly overlaps with that of other CTDs, leading to a high risk of misdiagnosis.<\/jats:italic><jats:italic>\u2022 Manifestations of MCTD are highly variable and potentially life-threatening, requiring continued immunomodulating treatment in most cases.<\/jats:italic><jats:italic>\u2022 A composite score based on SLEDAI-2\u00a0K and EUSTAR-AI measures could represent an easy applicable tool to monitor disease activity and treatment response.<\/jats:italic><\/jats:td>\n                    <\/jats:tr>\n                  <\/jats:tbody><\/jats:table><\/jats:table-wrap><\/jats:sec>","DOI":"10.1007\/s10067-022-06286-w","type":"journal-article","created":{"date-parts":[[2022,7,29]],"date-time":"2022-07-29T04:30:08Z","timestamp":1659069008000},"page":"3503-3511","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["The enigma of mixed connective tissue disease\u2014challenges in routine care"],"prefix":"10.1007","volume":"41","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-3962-8124","authenticated-orcid":false,"given":"Adrian","family":"Wanzenried","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Alexandru","family":"Garaiman","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Suzana","family":"Jordan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Oliver","family":"Distler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Britta","family":"Maurer","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2022,7,29]]},"reference":[{"issue":"2","key":"6286_CR1","doi-asserted-by":"publisher","first-page":"148","DOI":"10.1016\/0002-9343(72)90064-2","volume":"52","author":"GC Sharp","year":"1972","unstructured":"Sharp GC, Irvin WS, Tan EM, Gould RG, Holman HR (1972) Mixed connective tissue disease\u2013an apparently distinct rheumatic disease syndrome associated with a specific antibody to an extractable nuclear antigen (ENA). 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OD had consultancy relationship and\/or has received research funding from Abbvie, Actelion, Acceleron Pharma, Amgen, AnaMar, Baecon Discovery, Blade Therapeutics, Bayer, Boehringer Ingelheim, Catenion, Competitive Drug Development International Ltd., CSL Behring, ChemomAb, Curzion Pharmaceuticals, Ergonex, Galapagos NV, Glenmark Pharmaceuticals, GSK, Inventiva, Italfarmaco, iQone, iQvia, Lilly, medac, Medscape, Mitsubishi Tanabe Pharma, MSD, Novartis, Pfizer, Roche, Sanofi, Target Bio Science and UCB in the area of potential treatments of scleroderma and its complications. In addition, OD has a patent mir-29 for the treatment of systemic sclerosis issued (US8247389, EP2331143). BM had grant\/research support from AbbVie, Protagen and Novartis Biomedical Research and a consultancy relationship with Boehringer-Ingelheim as well as congress support from Pfizer, Roche, Actelion, mepha and MSD. In addition, BM has a patent mir-29 for the treatment of systemic sclerosis issued (US8247389, EP2331143).","order":4,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interests"}}]}}