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

Details

Serval ID
serval:BIB_D5B6E2510518
Type
Article: article from journal or magazin.
Collection
Publications
Institution
Title
Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis.
Journal
Arthritis & rheumatology
Author(s)
Sobanski V., Giovannelli J., Allanore Y., Riemekasten G., Airò P., Vettori S., Cozzi F., Distler O., Matucci-Cerinic M., Denton C., Launay D., Hachulla E.
Working group(s)
EUSTAR Collaborators
Contributor(s)
Guiducci S., Walker U., Kyburz D., Lapadula G., Iannone F., Maurer B., Jordan S., Becvar R., Sierakowsky S., Kowal Bielecka O., Cutolo M., Sulli A., Valentini G., Cuomo G., Siegert E., Rednic S., Nicoara I., Kahan A., Vlachoyiannopoulos P., Montecucco C., Caporali R., Stork J., Inanc M., Carreira P.E., Novak S., Czirják L., Varju C., Chizzolini C., Kucharz E.J., Kotulska A., Kopec-Medrek M., Widuchowska M., Rozman B., Mallia C., Coleiro B., Gabrielli A., Farge D., Wu C., Marjanovic Z., Faivre H., Hij D., Dhamadi R., Airò P., Hesselstrand R., Wollheim F., Wuttge D.M., Andréasson K., Martinovic D., Balbir-Gurman A., Braun-Moscovici Y., Trotta F., Lo Monaco A., Hunzelmann N., Pellerito R., Mauriziano O., Maria Bambara L., Caramaschi P., Morovic-Vergles J., Black C., Damjanov N., Henes J., Ortiz Santamaria V., Heitmann S., Krasowska D., Seidel M., Hasler P., Burkhardt H., Himsel A., Bajocchi G., Maria Nuova A.S., João Salvador M., Pereira Da Silva J.A., Stamenkovic B., Stankovic A., Francesco Selmi C., De Santis M., Marasini B., Tikly M., Ananieva L.P., Denisov L.N., Müller-Ladner U., Frerix M., Tarner I., Scorza R., Puppo F., Engelhart M., Strauss G., Nielsen H., Damgaard K., Szücs G., Szamosi S., Zea Mendoza A., de la Puente C., Sifuentes Giraldo W.A., Midtvedt Ø., Reiseter S., Garen T., Valesini G., Riccieri V., Maria Ionescu R., Opris D., Groseanu L., Wigley F.M., Sfrent Cornateanu R., Ionitescu R., Maria Gherghe A., Soare A., Gorga M., Bojinca M., Mihai C., Milicescu M., Sunderkötter C., Kuhn A., Sandorfi N., Schett G., Distler J.H., Beyer C., Meroni P., Ingegnoli F., Mouthon L., De Keyser F., Smith V., Paolo Cantatore F., Corrado A., Ullman S., Iversen L., Alberto von Mühlen C., Marilu Bohn J., Scussel Lonzetti L., Rosa Pozzi M., Eyerich K., Hein R., Knott E., Wiland P., Szmyrka-Kaczmarek M., Sokolik R., Morgiel E., Madej M., Houssiau F.A., Jose Alegre-Sancho J., Krummel-Lorenz B., Saar P., Aringer M., Günther C., Westhovens R., de Langhe E., Lenaerts J., Anic B., Baresic M., Mayer M., Üprus M., Otsa K., Yavuz S., Granel B., Cezar Radominski S., de Souza Müller C., Azevedo V.F., Jimenez S., Busquets J., Agachi S., Groppa L., Chiaburu L., Russu E., Popa S., Zenone T., Pileckyte M., Stebbings S., Highton J., Mathieu A., Vacca A., Sampaio-Barros P.D., Yoshinari N.H., Marangoni R.G., Martin P., Fuocco L., Stamp L., Chapman P., O'Donnell J., Solanki K., Doube A., Veale D., O'Rourke M., Loyo E., Li M., Abdel Atty Mohamed W.A., Rosato E., Amoroso A., Gigante A., Oksel F., Yargucu F., Tanaseanu C.M., Popescu M., Dumitrascu A., Tiglea I., Foti R., Chirieac R., Ancuta C., Furst D.E., Villiger P., Adler S., van Laar J., Kayser C., Eduardo C A.L., Fathi N., Hassanien M., de la Peña Lefebvre P.G., Rodriguez Rubio S., Valero Exposito M., Sibilia J., Chatelus E., Gottenberg J.E., Chifflot H., Litinsky I., Emery P., Buch M., Del Galdo F., Venalis A., Butrimiene I., Venalis P., Rugiene R., Karpec D., Ann Saketkoo L., Lasky J.A., Kerzberg E., Montoya F., Cosentino V., Limonta M., Luca Brucato A., Lupi E., Rosner I., Rozenbaum M., Slobodin G., Boulman N., Rimar D., Couto M., Spertini F., Ribi C., Buss G., Kahl S., Hsu V.M., Chen F., McCloskey D., Malveaux H., Louis Pasquali J., Martin T., Gorse A., Guffroy A., Poindron V.
ISSN
2326-5205 (Electronic)
ISSN-L
2326-5191
Publication state
Published
Issued date
09/2019
Peer-reviewed
Oui
Volume
71
Number
9
Pages
1553-1570
Language
english
Notes
Publication types: Evaluation Study ; Journal Article ; Research Support, Non-U.S. Gov't
Publication Status: ppublish
Abstract
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.
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.
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.
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.
Keywords
Adult, Aged, Autoantibodies/blood, Cluster Analysis, Databases, Factual, Europe/epidemiology, Female, Humans, Male, Middle Aged, Phenotype, Prognosis, Prospective Studies, 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, Severity of Illness Index
Pubmed
Web of science
Open Access
Yes
Create date
02/08/2021 16:49
Last modification date
03/08/2021 6:37
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