Produção Nacional
Revisado por pares
Mapping and predicting mortality from systemic sclerosis
2017; BMJ; Volume: 76; Issue: 11 Linguagem: Inglês
10.1136/annrheumdis-2017-211448
ISSN1468-2060
AutoresMuriel Elhaï, Christophe Meune, Marouane Boubaya, Jérôme Avouac, É. Hachulla, Alexandra Balbir‐Gurman, Gabriela Riemekasten, Paolo Airò, Beatriz Joven, Serena Vettori, Franco Cozzi, Susanne Ullman, László Czirják, Mohammed Tikly, Ulf Müller‐Ladner, Paola Caramaschi, Oliver Distler, Florenzo Iannone, Lidia P Ananieva, Roger Hesselstrand, Radim Bečvář, Armando Gabrielli, Nemanja Damjanov, Maria João Salvador, Valeria Riccieri, Carina Mihai, Gabriella Szűcs, Ulrich A. Walker, Nicolas Hunzelmann, Duška Martinović, Vanessa Smith, Carolina de Souza Müller, Carlomaurizio Montecucco, Daniela Opriș-Belinski, Francesca Ingegnoli, Panayiotis G. Vlachoyiannopoulos, Bojana Stamenković, Edoardo Rosato, Stefan Heitmann, Jörg H. W. Distler, Thierry Zénone, Matthias Seidel, Alessandra Vacca, Ellen De Langhe, Srđan Novak, Maurizio Cutolo, Luc Mouthon, Jörg Henes, Carlo Chizzolini, Carlos Alberto von Mühlen, Kamal Solanki, Simona Rednic, Lisa K. Stamp, Branimir Anić, Vera Ortiz-Santamaría, Maria De Santis, Şule Yavuz, Walter Alberto Sifuentes Giraldo, Emmanuel Chatelus, Jiří Štork, Jacob M. van Laar, Esthela Loyo, Paloma García de la Peña Lefebvre, Kilian Eyerich, Vanesa Cosentino, Juan José Alegre Sancho, Otylia Kowal‐Bielecka, Grégoire Rey, Marco Matucci‐Cerinic, Yannick Allanore,
Tópico(s)Dermatologic Treatments and Research
ResumoObjectives To determine the causes of death and risk factors in systemic sclerosis (SSc). Methods Between 2000 and 2011, we examined the death certificates of all French patients with SSc to determine causes of death. Then we examined causes of death and developed a score associated with all-cause mortality from the international European Scleroderma Trials and Research (EUSTAR) database. Candidate prognostic factors were tested by Cox proportional hazards regression model by single variable analysis, followed by a multiple variable model stratified by centres. The bootstrapping technique was used for internal validation. Results We identified 2719 French certificates of deaths related to SSc, mainly from cardiac (31%) and respiratory (18%) causes, and an increase in SSc-specific mortality over time. Over a median follow-up of 2.3 years, 1072 (9.6%) of 11 193 patients from the EUSTAR sample died, from cardiac disease in 27% and respiratory causes in 17%. By multiple variable analysis, a risk score was developed, which accurately predicted the 3-year mortality, with an area under the curve of 0.82. The 3-year survival of patients in the upper quartile was 53%, in contrast with 98% in the first quartile. Conclusion Combining two complementary and detailed databases enabled the collection of an unprecedented 3700 deaths, revealing the major contribution of the cardiopulmonary system to SSc mortality. We also developed a robust score to risk-stratify these patients and estimate their 3-year survival. With the emergence of new therapies, these important observations should help caregivers plan and refine the monitoring and management to prolong these patients’ survival.
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