Title: | Phenotypes Determined by Cluster Analysis and Their Survival in the Prospective European Scleroderma Trials and Research Cohort of Patients With Systemic Sclerosis |
Authors: | SOBANSKI, Vincent; GIOVANNELLI, Jonathan; ALLANORE, Yannick; RIEMEKASTEN, Gabriela; AIRO, Paolo; VETTORI, Serena; COZZI, Franco; DISTLER, Oliver; MATUCCI-CERINIC, Marco; DENTON, Christopher; LAUNAY, David; HACHULLA, Eric; CERINIC, Marco Matucci; GUIDUCCI, Serena; WALKER, Ulrich; KYBURZ, Diego; LAPADULA, Giovanni; IANNONE, Florenzo; DISTLER, Oliver; MAURER, Britta; JORDAN, Suzana; BECVAR, Radim; SIERAKOWSKY, Stanislaw; BIELECKA, Otylia Kowal; CUTOLO, Maurizio; SULLI, Alberto; VALENTINI, Gabriele; CUOMO, Giovanna; VETTORI, Serena; SIEGERT, Elise; REDNIC, Simona; NICOARA, Ileana; KAHAN, Andre; ALLANORE, Yannick; VLACHOYIANNOPOULOS, Panayiotis; MONTECUCCO, Carlo; CAPORALI, Roberto; STORK, Jiri; INANC, Murat; CARREIRA, Patricia E.; NOVAK, Srdan; CZIRJAK, Laszlo; VARJU, Cecilia; CHIZZOLINI, Carlo; KUCHARZ, Eugene J.; KOTULSKA, Anna; KOPEC-MEDREK, Magdalena; WIDUCHOWSKA, Malgorzata; COZZI, Franco; ROZMAN, Blaz; MALLIA, Carmel; COLEIRO, Bernard; GABRIELLI, Armando; FARGE, Dominique; WU, Chen; MARJANOVIC, Zora; FAIVRE, Helene; HIJ, Darin; DHAMADI, Roza; AIRO, Paolo; HESSELSTRAND, Roger; WOLLHEIM, Frank; WUTTGE, Dirk M.; ANDREASSON, Kristofer; MARTINOVIC, Duska; BALBIR-GURMAN, Alexandra; BRAUN-MOSCOVICI, Yolanda; TROTTA, Francesco; MONACO, Andrea Lo; HUNZELMANN, Nicolas; PELLERITO, Raffaele; BAMBARA, Lisa Maria; CARAMASCHI, Paola; MOROVIC-VERGLES, Jadranka; BLACK, Carol; DENTON, Christopher; DAMJANOV, Nemanja; HENES, Joerg; SANTAMARIA, Vera Ortiz; HEITMANN, Stefan; KRASOWSKA, Dorota; SEIDEL, Matthias; HASLER, Paul; BURKHARDT, Harald; HIMSEL, Andrea; BAJOCCHI, Gianluigi; NUOVA, Arcispedale Santa Maria; SALVADOR, Maria Joao; SILVA, Jose Antonio Pereira Da; STAMENKOVIC, Bojana; STANKOVIC, Aleksandra; SELMI, Carlo Francesco; SANTIS, Maria De; MARASINI, Bianca; TIKLY, Mohammed; ANANIEVA, Lidia P.; DENISOV, Lev N.; MUELLER-LADNER, Ulf; FRERIX, Marc; TARNER, Ingo; SCORZA, Raffaella; PUPPO, Francesco; ENGELHART, Merete; STRAUSS, Gitte; NIELSEN, Henrik; DAMGAARD, Kirsten; SZUCS, Gabriella; SZAMOSI, Szilvia; MENDOZA, Antonio Zea; PUENTE, Carlos de la; GIRALDO, Walter Alberto Sifuentes; MIDTVEDT, Oyvind; REISETER, Silje; GAREN, Torhild; HACHULLA, Eric; LAUNAY, David; VALESINI, Guido; RICCIERI, Valeria; IONESCU, Ruxandra Maria; OPRIS, Daniela; GROSEANU, Laura; WIGLEY, Fredrick M.; CORNATEANU, Roxana Sfrent; IONITESCU, Razvan; GHERGHE, Ana Maria; SOARE, Alina; GORGA, Marilena; BOJINCA, Mihai; MIHAI, Carina; MILICESCU, Mihaela; SUNDERKOETTER, Cord; KUHN, Annegret; SANDORFI, Nora; SCHETT, Georg; DISTLER, Joerg H. W.; BEYER, Christian; MERONI, Pierluigi; INGEGNOLI, Francesca; MOUTHON, Luc; KEYSER, Filip De; SMITH, Vanessa; CANTATORE, Francesco Paolo; CORRADO, Ada; ULLMAN, Susanne; IVERSEN, Line; MUEHLEN, Carlos Alberto von; BOHN, Jussara Marilu; LONZETTI, Lilian Scussel; POZZI, Maria Rosa; EYERICH, Kilian; HEIN, Ruediger; KNOTT, Elisabeth; WILAND, Piotr; SZMYRKA-KACZMAREK, Magdalena; SOKOLIK, Renata; MORGIEL, Ewa; MADEJ, Marta; HOUSSIAU, Frederic A.; ALEGRE-SANCHO, Juan Jose; KRUMMEL-LORENZ, Brigitte; SAAR, Petra; ARINGER, Martin; GUENTHER, Claudia; WESTHOVENS, Rene; LANGHE, Ellen de; LENAERTS, Jan; ANIC, Branimir; BARESIC, Marko; MAYER, Miroslav; UPRUS, Maria; OTSA, Kati; YAVUZ, Sule; GRANEL, Brigitte; RADOMINSKI, Sebastiao Cezar; MUELLER, Carolina de Souza; AZEVEDO, Valderilio Feijo; JIMENEZ, Sergio; BUSQUETS, Joanna; AGACHI, Svetlana; GROPPA, Liliana; CHIABURU, Lealea; RUSSU, Eugen; POPA, Sergei; ZENONE, Thierry; PILECKYTE, Margarita; STEBBINGS, Simon; HIGHTON, John; MATHIEU, Alessandro; VACCA, Alessandra; SAMPAIO-BARROS, Percival D.; YOSHINARI, Natalino H.; MARANGONI, Roberta G.; MARTIN, Patricia; FUOCCO, Luiza; STAMP, Lisa; CHAPMAN, Peter; O'DONNELL, John; SOLANKI, Kamal; DOUBE, Alan; VEALE, Douglas; O'ROURKE, Marie; LOYO, Esthela; LI, Mengtao; MOHAMED, Walid Ahmed Abdel Atty; ROSATO, Edoardo; AMOROSO, Antonio; GIGANTE, Antonietta; OKSEL, Fahrettin; YARGUCU, Figen; TANASEANU, Cristina-Mihaela; POPESCU, Monica; DUMITRASCU, Alina; TIGLEA, Isabela; FOTI, Rosario; CHIRIEAC, Rodica; ANCUTA, Codrina; FURST, Daniel E.; VILLIGER, Peter; ADLER, Sabine; LAAR, Jacob van; KAYSER, Cristiane; EDUARDO, Andrade Luis C.; FATHI, Nihal; HASSANIEN, Manal; LEFEBVRE, Paloma Garcia de la Pena; RUBIO, Silvia Rodriguez; EXPOSITO, Marta Valero; SIBILIA, Jean; CHATELUS, Emmanuel; GOTTENBERG, Jacques Eric; CHIFFLOT, Helene; LITINSKY, Ira; EMERY, Paul; BUCH, Maya; GALDO, Francesco Del; VENALIS, Algirdas; BUTRIMIENE, Irena; VENALIS, Paulius; RUGIENE, Rita; KARPEC, Diana; SAKETKOO, Lesley Ann; LASKY, Joseph A.; KERZBERG, Eduardo; MONTOYA, Fabiana; COSENTINO, Vanesa; LIMONTA, Massimiliano; BRUCATO, Antonio Luca; LUPI, Elide; ROSNER, Itzhak; ROZENBAUM, Michael; SLOBODIN, Gleb; BOULMAN, Nina; RIMAR, Doron; COUTO, Maura; SPERTINI, Francois; RIBI, Camillo; BUSS, Guillaume; KAHL, Sarah; HSU, Vivien M.; CHEN, Fei; MCCLOSKEY, Deborah; MALVEAUX, Halina; PASQUALI, Jean Louis; MARTIN, Thierry; GORSE, Audrey; GUFFROY, Aurelien; POINDRON, Vincent |
Citation: | ARTHRITIS & RHEUMATOLOGY, v.71, n.9, p.1553-1570, 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. |
Appears in Collections: | Artigos e Materiais de Revistas Científicas - FM/MCM Departamento de Clínica Médica - FM/MCM Artigos e Materiais de Revistas Científicas - HC/ICHC Instituto Central - HC/ICHC Artigos e Materiais de Revistas Científicas - LIM/17 LIM/17 - Laboratório de Investigação em Reumatologia |
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