Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis. Issue 9 (12th August 2019)

• Record Type: Journal Article
• Authors: Sobanski, Vincent; Giovannelli, Jonathan; Allanore, Yannick; Riemekasten, Gabriela; Airò, Paolo; Vettori, Serena; Cozzi, Franco; Distler, Oliver; Matucci‐Cerinic, Marco; Denton, Christopher; Launay, David; Hachulla, Eric
• Other Names: Guiducci Serena investigator.; Walker Ulrich investigator.; Kyburz Diego investigator.; Lapadula Giovanni investigator.; Iannone Florenzo investigator.; Maurer Britta investigator.; Jordan Suzana investigator.; Becvar Radim investigator.; Sierakowsky Stanislaw investigator.; Kowal Bielecka Otylia investigator.; Cutolo Maurizio investigator.; Sulli Alberto investigator.; Valentini Gabriele investigator.; Cuomo Giovanna investigator.; Siegert Elise investigator.; Rednic Simona investigator.; Nicoara Ileana investigator.; Kahan André investigator.; Vlachoyiannopoulos Panayiotis investigator.; Montecucco Carlo investigator.; Caporali Roberto investigator.; Stork Jiri investigator.; Inanc Murat investigator.; Carreira Patricia E. investigator.; Novak Srdan investigator.; Czirják László investigator.; Varju Cecilia investigator.; Chizzolini Carlo investigator.; Kucharz Eugene J. investigator.; Kotulska Anna investigator.; Kopec‐Medrek Magdalena investigator.; Widuchowska Malgorzata investigator.; Rozman Blaz investigator.; Mallia Carmel investigator.; Coleiro Bernard investigator.; Gabrielli Armando investigator.; Farge Dominique investigator.; Wu Chen investigator.; Marjanovic Zora investigator.; Faivre Helene investigator.; Hij Darin investigator.; Dhamadi Roza investigator.; Airò Paolo investigator.; Hesselstrand Roger investigator.; Wollheim Frank investigator.; Wuttge Dirk M investigator.; Andréasson Kristofer investigator.; Martinovic Duska investigator.; Balbir‐Gurman Alexandra investigator.; Braun‐Moscovici Yolanda investigator.; Trotta Francesco investigator.; Lo Monaco Andrea investigator.; Hunzelmann Nicolas investigator.; Pellerito Raffaele investigator.; Mauriziano Ospedale investigator.; Maria Bambara Lisa investigator.; Caramaschi Paola investigator.; Morovic‐Vergles Jadranka investigator.; Black Carol investigator.; Damjanov Nemanja investigator.; Henes Jörg investigator.; Ortiz Santamaria Vera investigator.; Heitmann Stefan investigator.; Krasowska Dorota investigator.; Seidel Matthias investigator.; Hasler Paul investigator.; Burkhardt Harald investigator.; Himsel Andrea investigator.; Bajocchi Gianluigi investigator.; Maria Nuova Arcispedale Santa investigator.; João Salvador Maria investigator.; Pereira Da Silva José Antonio investigator.; Stamenkovic Bojana investigator.; Stankovic Aleksandra investigator.; Francesco Selmi Carlo investigator.; De Santis Maria investigator.; Marasini Bianca investigator.; Tikly Mohammed investigator.; Ananieva Lidia P. investigator.; Denisov Lev N. investigator.; Müller‐Ladner Ulf investigator.; Frerix Marc investigator.; Tarner Ingo investigator.; Scorza Raffaella investigator.; Puppo Francesco investigator.; Engelhart Merete investigator.; Strauss Gitte investigator.; Nielsen Henrik investigator.; Damgaard Kirsten investigator.; Szücs Gabriella investigator.; Szamosi Szilvia investigator.; Zea Mendoza Antonio investigator.; de la Puente Carlos investigator.; Sifuentes Giraldo Walter Alberto investigator.; Midtvedt Øyvind investigator.; Reiseter Silje investigator.; Garen Torhild investigator.; Valesini Guido investigator.; Riccieri Valeria investigator.; Maria Ionescu Ruxandra investigator.; Opris Daniela investigator.; Groseanu Laura investigator.; Wigley Fredrick M. investigator.; Sfrent Cornateanu Roxana investigator.; Ionitescu Razvan investigator.; Maria Gherghe Ana investigator.; Soare Alina investigator.; Gorga Marilena investigator.; Bojinca Mihai investigator.; Mihai Carina investigator.; Milicescu Mihaela investigator.; Sunderkötter Cord investigator.; Kuhn Annegret investigator.; Sandorfi Nora investigator.; Schett Georg investigator.; Distler Jörg HW investigator.; Beyer Christian investigator.; Meroni Pierluigi investigator.; Ingegnoli Francesca investigator.; Mouthon Luc investigator.; De Keyser Filip investigator.; Smith Vanessa investigator.; Paolo Cantatore Francesco investigator.; Corrado Ada investigator.; Ullman Susanne investigator.; Iversen Line investigator.; Alberto von Mühlen Carlos investigator.; Marilu Bohn Jussara investigator.; Scussel Lonzetti Lilian investigator.; Rosa Pozzi Maria investigator.; Eyerich Kilian investigator.; Hein Rüdiger investigator.; Knott Elisabeth investigator.; Wiland Piotr investigator.; Szmyrka‐Kaczmarek Magdalena investigator.; Sokolik Renata investigator.; Morgiel Ewa investigator.; Madej Marta investigator.; Houssiau Frédéric A. investigator.; Jose Alegre‐Sancho Juan investigator.; Krummel‐Lorenz Brigitte investigator.; Saar Petra investigator.; Aringer Martin investigator.; Günther Claudia investigator.; Westhovens Rene investigator.; de Langhe Ellen investigator.; Lenaerts Jan investigator.; Anic Branimir investigator.; Baresic Marko investigator.; Mayer Miroslav investigator.; Üprus Maria investigator.; Otsa Kati investigator.; Yavuz Sule investigator.; Granel Brigitte investigator.; Cezar Radominski Sebastião investigator.; de Souza Müller Carolina investigator.; Azevedo Valderílio Feijó investigator.; Jimenez Sergio investigator.; Busquets Joanna investigator.; Agachi Svetlana investigator.; Groppa Liliana investigator.; Chiaburu Lealea investigator.; Russu Eugen investigator.; Popa Sergei investigator.; Zenone Thierry investigator.; Pileckyte Margarita investigator.; Stebbings Simon investigator.; Highton John investigator.; Mathieu Alessandro investigator.; Vacca Alessandra investigator.; Sampaio‐Barros Percival D. investigator.; Yoshinari Natalino H. investigator.; Marangoni Roberta G. investigator.; Martin Patrícia investigator.; Fuocco Luiza investigator.; Stamp Lisa investigator.; Chapman Peter investigator.; O'Donnell John investigator.; Solanki Kamal investigator.; Doube Alan investigator.; Veale Douglas investigator.; O'Rourke Marie investigator.; Loyo Esthela investigator.; Li Mengtao investigator.; Abdel Atty Mohamed Walid Ahmed investigator.; Rosato Edoardo investigator.; Amoroso Antonio investigator.; Gigante Antonietta investigator.; Oksel Fahrettin investigator.; Yargucu Figen investigator.; Tanaseanu Cristina‐Mihaela investigator.; Popescu Monica investigator.; Dumitrascu Alina investigator.; Tiglea Isabela investigator.; Foti Rosario investigator.; Chirieac Rodica investigator.; Ancuta Codrina investigator.; Furst Daniel E. investigator.; Villiger Peter investigator.; Adler Sabine investigator.; van Laar Jacob investigator.; Kayser Cristiane investigator.; Eduardo C. Andrade Luis investigator.; Fathi Nihal investigator.; Hassanien Manal investigator.; de la Peña Lefebvre Paloma García investigator.; Rodriguez Rubio Silvia investigator.; Valero Exposito Marta investigator.; Sibilia Jean investigator.; Chatelus Emmanuel investigator.; Gottenberg Jacques Eric investigator.; Chifflot Hélène investigator.; Litinsky Ira investigator.; Emery Paul investigator.; Buch Maya investigator.; Del Galdo Francesco investigator.; Venalis Algirdas investigator.; Butrimiene Irena investigator.; Venalis Paulius investigator.; Rugiene Rita investigator.; Karpec Diana investigator.; Ann Saketkoo Lesley investigator.; Lasky Joseph A. investigator.; Kerzberg Eduardo investigator.; Montoya Fabiana investigator.; Cosentino Vanesa investigator.; Limonta Massimiliano investigator.; Luca Brucato Antonio investigator.; Lupi Elide investigator.; Rosner Itzhak investigator.; Rozenbaum Michael investigator.; Slobodin Gleb investigator.; Boulman Nina investigator.; Rimar Doron investigator.; Couto Maura investigator.; Spertini François investigator.; Ribi Camillo investigator.; Buss Guillaume investigator.; Kahl Sarah investigator.; Hsu Vivien M. investigator.; Chen Fei investigator.; McCloskey Deborah investigator.; Malveaux Halina investigator.; Louis Pasquali Jean investigator.; Martin Thierry investigator.; Gorse Audrey investigator.; Guffroy Aurélien investigator.; Poindron Vincent investigator.;
• Is Part Of: Arthritis & rheumatology. Volume 71:Issue 9(2019)
• Page Start: 1553
• Page End: 1570
• Publication Date: 2019-08-12
• Subjects: Arthritis -- Periodicals; Rheumatism -- Periodicals; 616.72
• DOI: 10.1002/art.40906
• Languages: English
• ISSNs: 2326-5191

Abstract

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.