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Seventy adults were invited through a National Resource Centre for Rare Disorders. A study specific questionnaire including Brief Pain Inventory, Standardized Nordic Questionnaire, Fatigue Severity Scale, Hospital Anxiety &amp; Depression Scale, questions on physical activity, and disease burden was used. Fifty\u2010two persons participated, <jats:italic>n<\/jats:italic>\u00a0=\u00a034 with Loeys\u2013Dietz and <jats:italic>n<\/jats:italic>\u00a0=\u00a018 with vascular Ehlers\u2013Danlos syndrome, aged 18\u201368\u2009years, 58% women. Chronic pain (79%) and fatigue (58%) symptoms were common. Half developed pain during childhood\/adolescence. Sleep problems and high multi\u2010organ burden were significantly associated with <jats:italic>chronic pain (p<\/jats:italic>\u00a0=\u00a00.004<jats:italic>, p<\/jats:italic>\u00a0=\u00a00.014<jats:italic>)<\/jats:italic> and <jats:italic>high fatigue (p<\/jats:italic>\u2009&lt;\u20090.001<jats:italic>, p<\/jats:italic>\u2009&lt;\u20090.001<jats:italic>). Chronic pain<\/jats:italic> was associated with higher scores of <jats:italic>fatigue<\/jats:italic> (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.002). Higher scores of <jats:italic>fatigue<\/jats:italic> were associated with lower level of physical activity (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.014), higher cardiovascular burden (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.025), and higher symptoms of anxiety (<jats:italic>p<\/jats:italic>\u00a0=\u00a00.001). In this study, symptoms of chronic pain, fatigue, sleep problems, and disease burden seemed to mutually reinforce each other. Initiatives should consider interventions aimed at postponing the onset and reducing symptoms of pain, fatigue, and sleep problems and thus reduce the total disease burden at an early stage in patients with these complex conditions.<\/jats:p>","DOI":"10.1002\/ajmg.a.62858","type":"journal-article","created":{"date-parts":[[2022,6,10]],"date-time":"2022-06-10T10:48:31Z","timestamp":1654858111000},"page":"2605-2616","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":9,"title":["Pain and fatigue in adults with <scp>Loeys\u2013Dietz<\/scp> syndrome and vascular <scp>Ehlers\u2013Danlos<\/scp> syndrome, a questionnaire\u2010based study"],"prefix":"10.1002","volume":"188","author":[{"ORCID":"https:\/\/orcid.org\/0000-0002-1396-6715","authenticated-orcid":false,"given":"Johansen","family":"Heidi","sequence":"first","affiliation":[{"name":"TRS National Resource Centre for Rare Disorders Sunnaas Rehabilitation Hospital  Nesodden Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Velvin","family":"Gry","sequence":"additional","affiliation":[{"name":"TRS National Resource Centre for Rare Disorders Sunnaas Rehabilitation Hospital  Nesodden Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Ingeborg B.","family":"Lidal","sequence":"additional","affiliation":[{"name":"TRS National Resource Centre for Rare Disorders Sunnaas Rehabilitation Hospital  Nesodden Norway"},{"name":"Faculty of Health Oslo Metropolitan University (OsloMet)  Oslo Norway"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,6,10]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.a.36574"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/bja\/aen103"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.ejpain.2005.06.009"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/ajmg.c.31553"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(99)01308-2"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jpain.2007.09.006"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1136\/heartjnl-2014-306440"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.5664\/jcsm.27490"},{"issue":"2","key":"e_1_2_10_10_1","first-page":"129","article-title":"Pain assessment: Global use of the brief pain inventory","volume":"23","author":"Cleeland C. 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