{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,10,8]],"date-time":"2026-10-08T06:59:39Z","timestamp":1791442779531,"version":"4.3.3"},"reference-count":42,"publisher":"Wiley","issue":"9","license":[{"start":{"date-parts":[[2019,8,12]],"date-time":"2019-08-12T00:00:00Z","timestamp":1565568000000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by-nc-nd\/4.0\/"}],"content-domain":{"domain":["acrjournals.onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Arthritis &amp; Rheumatology"],"published-print":{"date-parts":[[2019,9]]},"abstract":"<jats:sec><jats:title>Objective<\/jats:title><jats:p>Systemic sclerosis (<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c (lc<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c) and diffuse cutaneous <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c (dc<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (<jats:styled-content style=\"fixed-case\">EUSTAR<\/jats:styled-content>) database includes data on a prospective cohort of <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of <jats:styled-content style=\"fixed-case\">EUSTAR<\/jats:styled-content> data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>A total of 11,318 patients were registered in the <jats:styled-content style=\"fixed-case\">EUSTAR<\/jats:styled-content> database, and 6,927 were included in the study. Twenty\u2010four clinical and serologic variables were used for clustering.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>Clustering analyses provided a first delineation of 2 clusters showing moderate stability. In an exploratory attempt, we further characterized 6 homogeneous groups that differed with regard to their clinical features, autoantibody profile, and mortality. Some groups resembled usual dc<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c or lc<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c prototypes, but others exhibited unique features, such as a majority of lc<jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients with a high rate of visceral damage and antitopoisomerase antibodies. Prognosis varied among groups and the presence of organ damage markedly impacted survival regardless of cutaneous involvement.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusion<\/jats:title><jats:p>Our findings suggest that restricting subsets of <jats:styled-content style=\"fixed-case\">SS<\/jats:styled-content>c patients to only those based on cutaneous involvement may not capture the complete heterogeneity of the disease. Organ damage and antibody profile should be taken into consideration when individuating homogeneous groups of patients with a distinct prognosis.<\/jats:p><\/jats:sec>","DOI":"10.1002\/art.40906","type":"journal-article","created":{"date-parts":[[2019,4,10]],"date-time":"2019-04-10T07:37:38Z","timestamp":1554881858000},"page":"1553-1570","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":130,"title":["Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis"],"prefix":"10.1002","volume":"71","author":[{"ORCID":"https:\/\/orcid.org\/0000-0003-3083-2441","authenticated-orcid":false,"given":"Vincent","family":"Sobanski","sequence":"first","affiliation":[{"name":"Universit\u00e9 Lille INSERM U995 LIRIC CHU Lille, and Referral Center for Rare Systemic Autoimmune Diseases North and North\u2010West of France  Lille 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