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Phenotypes determined by cluster analysis and their survival in the prospective European Scleroderma Trials and Research cohort of patients with systemic sclerosis
- Source :
- ARTHRITIS & RHEUMATOLOGY, Arthritis & rheumatology, vol. 71, no. 9, pp. 1553-1570, Arthritis & rheumatology, Hoboken : Wiley, 2019, vol. 71, iss. 9, p. 1553-1570, Arthritis & Rheumatology, Volume 71, Issue 9, Arthritis and Rheumatology, Vol. 71, No 9 (2019) pp. 1553-1570, r-FISABIO. Repositorio Institucional de Producción Científica, instname, r-FISABIO: Repositorio Institucional de Producción Científica, Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO)
- Publication Year :
- 2019
-
Abstract
- 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. ispartof: ARTHRITIS & RHEUMATOLOGY vol:71 issue:9 pages:1553-1570 ispartof: location:United States status: published
- Subjects :
- 0301 basic medicine
Male
BIOMEDICINE AND HEALTHCARE. Clinical Medical Sciences
Databases, Factual
systemic sclerosis
SUBSETS
Disease
Severity of Illness Index
Scleroderma
DISEASE
0302 clinical medicine
Medicine and Health Sciences
Immunology and Allergy
Cluster Analysis
CRITERIA
Prospective Studies
Prospective cohort study
skin and connective tissue diseases
integumentary system
BIOMEDICINA I ZDRAVSTVO. Kliničke medicinske znanosti
Adult
Aged
Autoantibodies/blood
Europe/epidemiology
Female
Humans
Middle Aged
Phenotype
Prognosis
Scleroderma, Diffuse/blood
Scleroderma, Diffuse/epidemiology
Scleroderma, Diffuse/pathology
Scleroderma, Limited/blood
Scleroderma, Limited/epidemiology
Scleroderma, Limited/pathology
Scleroderma, Systemic/blood
Scleroderma, Systemic/epidemiology
Scleroderma, Systemic/pathology
Connective tissue disease
ddc
Europe
MANIFESTATIONS
Cohort
Life Sciences & Biomedicine
medicine.medical_specialty
Immunology
PROFILE
CLASSIFICATION
03 medical and health sciences
Rheumatology
Scleroderma, Limited
Internal medicine
Severity of illness
medicine
Autoantibodies
030203 arthritis & rheumatology
Science & Technology
Scleroderma, Systemic
business.industry
Autoantibody
Systemic sclerosis (SSc)
medicine.disease
030104 developmental biology
Scleroderma, Diffuse
business
Subjects
Details
- Language :
- English
- ISSN :
- 15531570, 23265191, and 23265205
- Database :
- OpenAIRE
- Journal :
- ARTHRITIS & RHEUMATOLOGY, Arthritis & rheumatology, vol. 71, no. 9, pp. 1553-1570, Arthritis & rheumatology, Hoboken : Wiley, 2019, vol. 71, iss. 9, p. 1553-1570, Arthritis & Rheumatology, Volume 71, Issue 9, Arthritis and Rheumatology, Vol. 71, No 9 (2019) pp. 1553-1570, r-FISABIO. Repositorio Institucional de Producción Científica, instname, r-FISABIO: Repositorio Institucional de Producción Científica, Fundación para el Fomento de la Investigación Sanitaria y Biomédica de la Comunitat Valenciana (FISABIO)
- Accession number :
- edsair.doi.dedup.....6131d10af0499850dede6c961d2bbbf9