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: Kyburz Diego investigator.; Maurer Britta investigator.; Jordan Suzana investigator.; Bielecka Otylia Kowal investigator.; Vlachoyiannopoulos Panayiotis investigator.; Montecucco Carlo investigator.; Stork Jiri investigator.; Inanc Murat investigator.; Morovic‐Vergles Jadranka investigator.; Hasler Paul investigator.; Bajocchi Gianluigi investigator.; Selmi Carlo Francesco investigator.; De Santis Maria investigator.; Marasini Bianca investigator.; Puppo Francesco investigator.; Szücs Gabriella investigator.; Szamosi Szilvia investigator.; Giraldo Walter Alberto Sifuentes investigator.; Garen Torhild investigator.; Wigley Fredrick M. investigator.; Mihai Carina investigator.; Milicescu Mihaela investigator.; Sunderkötter Cord investigator.; Kuhn Annegret investigator.; Sandorfi Nora investigator.; Ullman Susanne investigator.; Iversen Line investigator.; von Mühlen Carlos Alberto investigator.; Bohn Jussara Marilu investigator.; Lonzetti Lilian Scussel investigator.; Houssiau Frédéric A. investigator.; Alegre‐Sancho Juan Jose investigator.; Üprus Maria investigator.; Otsa Kati investigator.; Yavuz Sule investigator.; Granel Brigitte investigator.; Jimenez Sergio investigator.; Busquets Joanna investigator.; Pileckyte Margarita 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.; Mohamed Walid Ahmed Abdel Atty investigator.; Oksel Fahrettin investigator.; Yargucu Figen investigator.; Furst Daniel E. investigator.; van Laar Jacob investigator.; Kayser Cristiane investigator.; Eduardo C. Andrade Luis investigator.; Fathi Nihal investigator.; Emery Paul investigator.; Buch Maya investigator.; Del Galdo Francesco investigator.; Rosner Itzhak investigator.; Rozenbaum Michael investigator.; Slobodin Gleb investigator.; Boulman Nina investigator.; Rimar Doron investigator.; Couto Maura investigator.; Kahl Sarah investigator.; Hsu Vivien M. investigator.; Chen Fei investigator.; McCloskey Deborah investigator.; Malveaux Halina 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.