A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: A EUSTAR prospective study

Muriel Elhai, Jérôme Avouac, Ulrich A. Walker, Marco Matucci-Cerinic, Gabriela Riemekasten, Paolo Airò, Eric Hachulla, Gabriele Valentini, Patricia E. Carreira, Franco Cozzi, Alexandra Balbir Gurman, Yolanda Braun-Moscovici, Nemanja Damjanov, Lidia P. Ananieva, Raffaella Scorza, Sergio Jimenez, Joanna Busquets, Mengtao Li, Ulf Müller-Ladner, André KahanOliver Distler, Yannick Allanore, Serena Guiducci, Alan Tyndall, Giovanni Lapadula, Florenzo Iannone, Radim Becvar, Stanislaw Sierakowsky, Otylia Kowal Bielecka, Maurizio Cutolo, Alberto Sulli, Giovanna Cuomo, Serena Vettori, Simona Rednic, Ileana Nicoara, P. Vlachoyiannopoulos, C. Montecucco, Roberto Caporali, Srdan Novak, László Czirják, Cecilia Varju, Carlo Chizzolini, Eugene J. Kucharz, Anna Kotulska, Magdalena Kopec-Medrek, Malgorzata Widuchowska, Blaz Rozman, Carmel Mallia, Bernard Coleiro, Armando Gabrielli, Dominique Farge, Adrian Hij, Roger Hesselstrand, Agneta Scheja, Frank Wollheim, Duska Martinovic, M. Govoni, Andrea Lo Monaco, Nicolas Hunzelmann, Raffaele Pellerito, Lisa Maria Bambara, Paola Caramaschi, Carol Black, Christopher Denton, Jörg Henes, Vera Ortiz Santamaria, Stefan Heitmann, Dorota Krasowska, Matthias Seidel, Mara Oleszowsky, Harald Burkhardt, Andrea Himsel, Maria J. Salvador, Bojana Stamenkovic, Aleksandra Stankovic, Mohammed Tikly, Maya N. Starovoytova, Merete Engelhart, Gitte Strauss, Henrik Nielsen, Kirsten Damgaard, Gabriella Szücs, Antonio Zea Mendoza, Carlos De La Puente Buijdos, Walter A.Sifuentes Giraldo, Øyvind Midtvedt, Torhild Garen, David Launay, Guido Valesini, Valeria Riccieri, Ruxandra Maria Ionescu, Daniela Opris, Laura Groseanu, Fredrick M. Wigley, Carmen M. Mihai, Roxana Sfrent Cornateanu, Razvan Ionitescu, Ana Maria Gherghe, Marilena Gorga, Rucsandra Dobrota, Mihai Bojinca, Georg Schett, Jörg H.W. Distler, Pierluigi Meroni, Silvana Zeni, Luc Mouthon, Filip De Keyser, Vanessa Smith, Francesco P. Cantatore, Ada Corrado, Susanne Ullman, Line Iversen, Maria R. Pozzi, Kilian Eyerich, Rüdiger Hein, Elisabeth Knott, Jacek Szechinski, Piotr Wiland, Magdalena Szmyrka-Kaczmarek, Renata Sokolik, Ewa Morgiel, Brigitte Krummel-Lorenz, Petra Saar, Martin Aringer, Claudia Günther, Branimir Anic, Marko Baresic, Miroslav Mayer, Sebastião C. Radominski, Carolina De Souza Müller, Valderílio F. Azevedo, Svetlana Agachi, Liliana Groppa, Lealea Chiaburu, Eugen Russu, Thierry Zenone, Simon Stebbings, John Highton, Lisa Stamp, Peter Chapman, Murray Baron, John O'Donnell, Kamal Solanki, Alan Doube, Douglas Veale, Marie O'Rourke, Esthela Loyo, Edoardo Rosato, Simonetta Pisarri, Cristina Mihaela Tanaseanu, Monica Popescu, Alina Dumitrascu, Isabela Tiglea, Rodica Chirieac, Codrina Ancuta, Daniel E. Furst, Suzanne Kafaja, Paloma García De La Peña Lefebvre, Silvia Rodriguez Rubio, Marta Valero Exposito, Jean Sibilia, Emmanuel Chatelus, Jacques Eric Gottenberg, Hélène Chifflot, Ira Litinsky, Algirdas Venalis, Irena Butrimiene, Paulius Venalis, Rita Rugiene, Diana Karpec, Eduardo Kerzberg, Fabiana Montoya, Vanesa Cosentino

Research output: Article

46 Citations (Scopus)

Abstract

Objectives. In agreement with other autoimmune diseases, systemic sclerosis (SSc) is associated with a strong sex bias. However, unlike lupus, the effects of sex on disease phenotype and prognosis are poorly known. Therefore, we aimed to determine sex effects on outcomes. Method. We performed a prospective observational study using the latest 2013 data extract from the EULAR scleroderma trials and research (EUSTAR) cohort. We looked at (i) sex influence on disease characteristics at baseline and (ii) then focused on patients with at least 2 years of follow-up to estimate the effects of sex on disease progression and survival. Results. 9182 patients with SSc were available (1321 men) for the baseline analyses. In multivariate analysis, male sex was independently associated with a higher risk of diffuse cutaneous subtype (OR: 1.68, (1.45 to 1.94); p<0.001), a higher frequency of digital ulcers (OR: 1.28 (1.11 to 1.47); p<0.001) and pulmonary hypertension (OR: 3.01 (1.47 to 6.20); p<0.003). In the longitudinal analysis (n=4499), after a mean follow-up of 4.9 (±2.7) years, male sex was predictive of new onset of pulmonary hypertension (HR: 2.66 (1.32 to 5.36); p=0.006) and heart failure (HR: 2.22 (1.06 to 4.63); p=0.035). 908 deaths were recorded, male sex predicted deaths of all origins (HR: 1.48 (1.19 to 1.84); p<0.001), but did not significantly account for SSc-related deaths. Conclusions. Although more common in women, SSc appears as strikingly more severe in men. Our results obtained through the largest worldwide database demonstrate a higher risk of severe cardiovascular involvement in men. These results raise the point of including sex in the management and the decisionmaking process.

Original languageEnglish
Pages (from-to)163-169
Number of pages7
JournalAnnals of the rheumatic diseases
Volume75
Issue number1
DOIs
Publication statusPublished - jan. 1 2016

ASJC Scopus subject areas

  • Immunology and Allergy
  • Rheumatology
  • Immunology
  • Biochemistry, Genetics and Molecular Biology(all)

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    Elhai, M., Avouac, J., Walker, U. A., Matucci-Cerinic, M., Riemekasten, G., Airò, P., Hachulla, E., Valentini, G., Carreira, P. E., Cozzi, F., Gurman, A. B., Braun-Moscovici, Y., Damjanov, N., Ananieva, L. P., Scorza, R., Jimenez, S., Busquets, J., Li, M., Müller-Ladner, U., ... Cosentino, V. (2016). A gender gap in primary and secondary heart dysfunctions in systemic sclerosis: A EUSTAR prospective study. Annals of the rheumatic diseases, 75(1), 163-169. https://doi.org/10.1136/annrheumdis-2014-206386