2 research outputs found
Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis
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
Phenotypes determined by cluster analysis and their survival in the prospective european scleroderma trials and research cohort of patients with systemic sclerosis
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