Mapping and predicting mortality from systemic sclerosis

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Mapping and predicting mortality from systemic sclerosis

Elhai, Muriel (author): Paris Descartes University

Meune, Christophe (author): Sorbonne Paris Nord University

Boubaya, Marouane (author): Sorbonne Paris Nord University

Avouac, Jérôme (author): Paris Descartes University

Hachulla, Eric (author): University of Lille

Balbir-Gurman, Alexandra (author): Technion - Israel Institute of Technology

Riemekasten, Gabriela (author): University of Lübeck

Airò, Paolo (author): Spedali Civili Di Brescia

Joven, Beatriz (author): 12 de Octubre University Hospital

Vettori, Serena (author): University of Campania Luigi Vanvitelli

Cozzi, Franco (author): University of Padova

Ullman, Susanne (author): Copenhagen University Hospital

Czirják, László (author): University of Pécs

Tikly, Mohammed (author)

Müller-Ladner, Ulf (author): Justus Liebig University Giessen

Caramaschi, Paola (author): University of Verona

Distler, Oliver (author): University Hospital of Zurich

Iannone, Florenzo (author): University of Bari Aldo Moro

Ananieva, Lidia P. (author)

Hesselstrand, Roger (author): Lund University,Lunds universitet,Reumatologi och molekylär skelettbiologi,Sektion III,Institutionen för kliniska vetenskaper, Lund,Medicinska fakulteten,Rheumatology,Section III,Department of Clinical Sciences, Lund,Faculty of Medicine

Becvar, Radim (author): Charles University in Prague

Gabrielli, Armando (author): Marche Polytechnic University

Damjanov, Nemanja (author): University of Belgrade

Salvador, Maria Joao (author)

Riccieri, Valeria (author): Sapienza University of Rome

Mihai, Carina (author): Carol Davila University of Medicine and Pharmacy

Szücs, Gabriella (author)

Walker, Ulrich A. (author): University Hospital Basel

Hunzelmann, Nicolas (author): University Hospital of Cologne

Martinovic, Duska (author): Clinical Hospital Split

Smith, Vanessa (author): Ghent University

Müller, Carolina De Souza (author): Federal University of Paraná

Montecucco, Carlo Maurizio (author): Policlinico San Matteo Pavia Fondazione

Opris, Daniela (author): Carol Davila University of Medicine and Pharmacy

Ingegnoli, Francesca (author): University of Milan

Vlachoyiannopoulos, Panayiotis G. (author): National and Kapodistrian University of Athens

Stamenkovic, Bojana (author)

Rosato, Edoardo (author): Sapienza University of Rome

Heitmann, Stefan (author)

Distler, Jörg H W (author): University Hospital Erlangen

Zenone, Thierry (author): Centre Hospitalier de Valence

Seidel, Matthias (author): University Hospital Bonn

Vacca, Alessandra (author)

De Langhe, Ellen (author): University Hospitals Leuven

Novak, Srdan (author)

Cutolo, Maurizio (author): University of Genoa

Mouthon, Luc (author): Cochin Hospital

Henes, Jörg (author): University Hospital of Tubingen

Chizzolini, Carlo (author): Geneva University Hospital

von Mühlen, Carlos Alberto (author)

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(creator_code:org_t)

2017-08-23
2017
English 9 s.
In: Annals of the Rheumatic Diseases. - : BMJ. - 0003-4967 .- 1468-2060. ; 76:11, s. 1897-1905

Related links:: http://dx.doi.org/10...; show more...; https://lirias.kuleu...; https://lup.lub.lu.s...; https://doi.org/10.1...; show less...

Journal article (peer-reviewed)

Abstract Subject headings

Objectives 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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By the author/editor: Elhai, Muriel; Meune, Christoph ...; Boubaya, Marouan ...; Avouac, Jérôme; Hachulla, Eric; Balbir-Gurman, A ...; show more...; Riemekasten, Gab ...; Airò, Paolo; Joven, Beatriz; Vettori, Serena; Cozzi, Franco; Ullman, Susanne; Czirják, László; Tikly, Mohammed; Müller-Ladner, U ...; Caramaschi, Paol ...; Distler, Oliver; Iannone, Florenz ...; Ananieva, Lidia ...; Hesselstrand, Ro ...; Becvar, Radim; Gabrielli, Arman ...; Damjanov, Nemanj ...; Salvador, Maria ...; Riccieri, Valeri ...; Mihai, Carina; Szücs, Gabriella; Walker, Ulrich A ...; Hunzelmann, Nico ...; Martinovic, Dusk ...; Smith, Vanessa; Müller, Carolina ...; Montecucco, Carl ...; Opris, Daniela; Ingegnoli, Franc ...; Vlachoyiannopoul ...; Stamenkovic, Boj ...; Rosato, Edoardo; Heitmann, Stefan; Distler, Jörg H ...; Zenone, Thierry; Seidel, Matthias; Vacca, Alessandr ...; De Langhe, Ellen; Novak, Srdan; Cutolo, Maurizio; Mouthon, Luc; Henes, Jörg; Chizzolini, Carl ...; von Mühlen, Carl ...; show less...

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MEDICAL AND HEALTH SCIENCES: MEDICAL AND HEAL ...; and Clinical Medicin ...; and Cardiac and Card ...

Articles in the publication: Annals of the Rh ...

By the university: Lund University

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Mapping and predicting mortality from systemic sclerosis

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