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Bispebjerg Hospital - a part of Copenhagen University Hospital

Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis

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  1. Incidence and risk factors for gangrene in patients with systemic sclerosis from the EUSTAR cohort

    Research output: Contribution to journalJournal articleResearchpeer-review

  2. ACE inhibitors in SSc patients display a risk factor for scleroderma renal crisis-a EUSTAR analysis

    Research output: Contribution to journalJournal articleResearchpeer-review

  3. Outcomes of limited cutaneous systemic sclerosis patients: Results on more than 12,000 patients from the EUSTAR database

    Research output: Contribution to journalJournal articleResearchpeer-review

  • EUSTAR Collaborators
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OBJECTIVE: Systemic sclerosis (SSc) is a heterogeneous connective tissue disease that is typically subdivided into limited cutaneous SSc (lcSSc) and diffuse cutaneous SSc (dcSSc) depending on the extent of skin involvement. This subclassification may not capture the entire variability of clinical phenotypes. The European Scleroderma Trials and Research (EUSTAR) database includes data on a prospective cohort of SSc patients from 122 European referral centers. This study was undertaken to perform a cluster analysis of EUSTAR data to distinguish and characterize homogeneous phenotypes without any a priori assumptions, and to examine survival among the clusters obtained.

METHODS: A total of 11,318 patients were registered in the EUSTAR database, and 6,927 were included in the study. Twenty-four clinical and serologic variables were used for clustering.

RESULTS: 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 dcSSc or lcSSc prototypes, but others exhibited unique features, such as a majority of lcSSc 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.

CONCLUSION: Our findings suggest that restricting subsets of SSc 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.

Original languageEnglish
JournalArthritis & rheumatology
Issue number9
Pages (from-to)1553-1570
Number of pages18
Publication statusPublished - Sep 2019

    Research areas

  • Adult, Aged, Autoantibodies/blood, Cluster Analysis, Databases, Factual, Europe/epidemiology, Female, Humans, Male, Middle Aged, Phenotype, Prognosis, Prospective Studies, Scleroderma, Diffuse/blood, Scleroderma, Limited/blood, Scleroderma, Systemic/blood, Severity of Illness Index

ID: 59236463