{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,20]],"date-time":"2026-10-20T21:26:19Z","timestamp":1792531579272,"version":"4.5.0"},"reference-count":20,"publisher":"Wiley","issue":"8","license":[{"start":{"date-parts":[[2019,7,3]],"date-time":"2019-07-03T00:00:00Z","timestamp":1562112000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis Care &amp;amp; Research"],"published-print":{"date-parts":[[2019,8]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Assessment of Raynaud's phenomenon (<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content>) in systemic sclerosis (<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c) is reliant on self\u2010report. The Raynaud's Condition Score (<jats:styled-content style=\"fixed-case\">RCS<\/jats:styled-content>) diary assumes discrete episodic <jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content> attacks, although not all <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients identify with this paradigm. We investigated the clinical associations of <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content> symptom characteristics and the evolution of <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content> symptoms with disease progression.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A cross\u2010sectional study at <jats:styled-content style=\"fixed-case\">UK<\/jats:styled-content> and <jats:styled-content style=\"fixed-case\">US<\/jats:styled-content> sites captured digital color changes of <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content> and patients\u2019 ability to identify with diagrammatic representations (and descriptive stems) of 4 distinct theoretical <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content> patterns (progressing severity A through D) reflecting progressively severe <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content> experiences. <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content> self\u2010management and symptom evolution were explored. Patient demographics, the clinical phenotype, the Scleroderma Health Assessment Questionnaire (<jats:styled-content style=\"fixed-case\">SHAQ<\/jats:styled-content>), the 2\u2010week <jats:styled-content style=\"fixed-case\">RCS<\/jats:styled-content> diary, and patient and physician global assessments were collected.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>We enrolled 107 patients with <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c (with questionnaires returned by 94). A higher number of self\u2010reported digital color changes of <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content> were associated with increased <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content> symptom severity but not with the <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c clinical phenotype. Patients could identify with distinct patterns of <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content>. These patterns were associated with disease duration, global disease severity, and conceptually linked physician and patient assessments of peripheral vascular severity (e.g., <jats:styled-content style=\"fixed-case\">SHAQ RP<\/jats:styled-content> subscale and <jats:styled-content style=\"fixed-case\">RCS<\/jats:styled-content> diary parameters), but not with conceptually unrelated outcomes (e.g., <jats:styled-content style=\"fixed-case\">SHAQ<\/jats:styled-content> breathing subscale). <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content> characteristics and symptom severity evolve during the disease course.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Patients identify with distinct patterns of <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content> that may relate to progression of the obliterative microangiopathy of <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c. Difficulty distinguishing discrete <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content> attacks from persistent digital ischemia in patients with advanced <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c could influence diary\u2010based approaches to assessing <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c\u2010<jats:styled-content style=\"fixed-case\">RP<\/jats:styled-content>, with implications for future clinical trials.<\/jats:p><\/jats:sec>","DOI":"10.1002\/acr.23729","type":"journal-article","created":{"date-parts":[[2018,8,22]],"date-time":"2018-08-22T11:57:04Z","timestamp":1534939024000},"page":"1119-1126","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":37,"title":["Evolving Symptom Characteristics of Raynaud's Phenomenon in Systemic Sclerosis and Their Association With Physician and Patient\u2010Reported Assessments of Disease Severity"],"prefix":"10.1002","volume":"71","author":[{"given":"John D.","family":"Pauling","sequence":"first","affiliation":[{"name":"Royal National Hospital for Rheumatic Diseases Royal United Hospitals and University of Bath  Bath UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Elizabeth","family":"Reilly","sequence":"additional","affiliation":[{"name":"Royal National Hospital for Rheumatic Diseases Royal United Hospitals and University of Bath  Bath UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Theresa","family":"Smith","sequence":"additional","affiliation":[{"name":"University of Bath  Bath UK"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-5472-3840","authenticated-orcid":false,"given":"Tracy M.","family":"Frech","sequence":"additional","affiliation":[{"name":"Salt Lake Regional Veterans Affairs Medical Center and University of Utah  Salt Lake City"}],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2019,7,3]]},"reference":[{"key":"e_1_2_6_2_1","doi-asserted-by":"publisher","DOI":"10.1056\/NEJMra1507638"},{"key":"e_1_2_6_3_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/keq310"},{"key":"e_1_2_6_4_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/key026"},{"key":"e_1_2_6_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/acr.23475"},{"key":"e_1_2_6_6_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jaut.2014.01.020"},{"key":"e_1_2_6_7_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.38098"},{"key":"e_1_2_6_8_1","doi-asserted-by":"publisher","DOI":"10.1093\/rheumatology\/32.5.357"},{"key":"e_1_2_6_9_1","first-page":"454","article-title":"Diagnosis of Raynaud's phenomenon assisted by color charts","volume":"15","author":"Maricq HR","year":"1988","journal-title":"J Rheumatol"},{"key":"e_1_2_6_10_1","doi-asserted-by":"publisher","DOI":"10.1155\/2015\/371960"},{"key":"e_1_2_6_11_1","doi-asserted-by":"publisher","DOI":"10.1002\/1529-0131(199804)41:4<670::AID-ART14>3.0.CO;2-I"},{"key":"e_1_2_6_12_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.40242"},{"key":"e_1_2_6_13_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.30195"},{"issue":"106","key":"e_1_2_6_14_1","first-page":"184","article-title":"Patient participation in patient\u2010reported outcome instrument development in systemic sclerosis","volume":"35","author":"Pauling JD","year":"2017","journal-title":"Clin Exp Rheumatol"},{"key":"e_1_2_6_15_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.40481"},{"key":"e_1_2_6_16_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.40457"},{"key":"e_1_2_6_17_1","doi-asserted-by":"publisher","DOI":"10.3899\/jrheum.141437"},{"key":"e_1_2_6_18_1","doi-asserted-by":"publisher","DOI":"10.1002\/art.1780401110"},{"key":"e_1_2_6_19_1","first-page":"202","article-title":"Scleroderma (systemic sclerosis): classification, subsets and pathogenesis","volume":"15","author":"LeRoy EC","year":"1988","journal-title":"J Rheumatol"},{"key":"e_1_2_6_20_1","doi-asserted-by":"publisher","DOI":"10.1002\/1529-0131(200002)43:2<444::AID-ANR27>3.0.CO;2-G"},{"key":"e_1_2_6_21_1","doi-asserted-by":"publisher","DOI":"10.1177\/2397198318774307"}],"container-title":["Arthritis Care &amp; 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