Sobanski V, Giovannelli J, Allanore Y, Riemekasten G, Airo P, Vettori S, Cozzi F, Distler O, Matucci-Cerinic M, Denton C, Launay D, Hachulla E, Cerinic MM, Guiducci S, Walker U, Kyburz D, Lapadula G, Iannone F, Distler O, Maurer B, Jordan S, Becvar R, Sierakowsky S, Bielecka OK, Cutolo M, Sulli A, Valentini G, Cuomo G, Vettori S, Siegert E, Rednic S, Nicoara I, Kahan A, Allanore Y, Vlachoyiannopoulos P, Montecucco C, Caporali R, Stork J, Inanc M, Carreira PE, Novak S, Czirjak L, Varju C, Chizzolini C, Kucharz EJ, Kotulska A, Kopec-Medrek M, Widuchowska M, Cozzi F, Rozman B, Mallia C, Coleiro B, Gabrielli A, Farge D, Wu C, Marjanovic Z, Faivre H, Hij D, Dhamadi R, Airo P, Hesselstrand R, Wollheim F, Wuttge DM, Andreasson K, Martinovic D, Balbir-Gurman A, Braun-Moscovici Y, Trotta F, Lo Monaco A, Hunzelmann N, Pellerito R, Bambara LM, Caramaschi P, Morovic-Vergles J, Black C, Denton C, Damjanov N, Henes J, Ortiz Santamaria V, Heitmann S, Krasowska D, Seidel M, Hasler P, Burkhardt H, Himsel A, Bajocchi G, Nuova ASM, Salvador MJ, Pereira Da Silva JA, Stamenkovic B, Stankovic A, Selmi CF, De Santis M, Marasini B, Tikly M, Ananieva LP, Denisov LN, Mueller-Ladner U, Frerix M, Tarner I, Scorza R, Puppo F, Engelhart M, Strauss G, Nielsen H, Damgaard K, Szucs G, Szamosi S, Zea Mendoza A, De La Puente C, Sifuentes Giraldo WA, Midtvedt O, Reiseter S, Garen T, Hachulla E, Launay D, Valesini G, Riccieri V, Ionescu RM, Opris D, Groseanu L, Wigley FM, Cornateanu RS, Ionitescu R, Gherghe AM, Soare A, Gorga M, Bojinca M, Mihai C, Milicescu M, Sunderkoetter C, Kuhn A, Sandorfi N, Schett G, Distler J, Beyer C, Meroni P, Ingegnoli F, Mouthon L, De Keyser F, Smith V, Cantatore FP, Corrado A, Ullman S, Iversen L, Von Muehlen CA, Bohn JM, Lonzetti LS, Pozzi MR, Eyerich K, Hein R, Knott E, Wiland P, Szmyrka-Kaczmarek M, Sokolik R, Morgiel E, Madej M, Houssiau FA, 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, Uprus M, Otsa K, Yavuz S, Granel B, Radominski SC, Mueller CDS, Azevedo VF, 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 PD, Yoshinari NH, Marangoni RG, Martin P, Fuocco L, Stamp L, Chapman P, O'Donnell J, Solanki K, Doube A, Veale D, O'Rourke M, Loyo E, Li M, Mohamed WAAA, Rosato E, Amoroso A, Gigante A, Oksel F, Yargucu F, Tanaseanu CM, Popescu M, Dumitrascu A, Tiglea I, Foti R, Chirieac R, Ancuta C, Furst DE, Villiger P, Adler S, Van Laar J, Kayser C, Eduardo ALC, Fathi N, Hassanien M, De La Pena Lefebvre PG, Rodriguez Rubio S, Valero Exposito M, Sibilia J, Chatelus E, Gottenberg JE, Chifflot H, Litinsky I, Emery P, Buch M, Del Galdo F, Venalis A, Butrimiene I, Venalis P, Rugiene R, Karpec D, Saketkoo LA, Lasky JA, Kerzberg E, Montoya F, Cosentino V, Limonta M, Brucato AL, Lupi E, Rosner I, Rozenbaum M, Slobodin G, Boulman N, Rimar D, Couto M, Spertini F, Ribi C, Buss G, Kahl S, Hsu VM, Chen F, Mccloskey D, Malveaux H, Pasquali JL, Martin T, Gorse A, Guffroy A, Poindron V (2019)
Publication Type: Journal article
Publication year: 2019
DOI: 10.1002/art.40906
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.
APA:
Sobanski, V., Giovannelli, J., Allanore, Y., Riemekasten, G., Airo, P., Vettori, S.,... Poindron, V. (2019). Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis. Arthritis and Rheumatology. https://dx.doi.org/10.1002/art.40906
MLA:
Sobanski, Vincent, et al. "Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis." Arthritis and Rheumatology (2019).
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