Mapping and predicting mortality from systemic sclerosis

Muriel Elhai; Christophe Meune; Marouane Boubaya; Jérôme Avouac; Eric 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 Becvar; Armando Gabrielli; Nemanja Damjanov; Maria J Salvador; Valeria Riccieri; Carina Mihai; Gabriella Szücs; Ulrich A Walker; Nicolas Hunzelmann; Duska Martinovic; Vanessa Smith; Carolina de Souza Müller; Carlo Maurizio Montecucco; Daniela Opris; Francesca Ingegnoli; Panayiotis G Vlachoyiannopoulos; Bojana Stamenkovic; Edoardo Rosato; Stefan Heitmann; Jörg H W Distler; Thierry Zenone; Matthias Seidel; Alessandra Vacca; Ellen De Langhe; Srdan Novak; Maurizio Cutolo; Luc Mouthon; Jörg Henes; Carlo Chizzolini; Carlos Alberto von Mühlen; EUSTAR group

doi:10.1136/annrheumdis-2017-211448

Mapping and predicting mortality from systemic sclerosis

Muriel Elhai, Christophe Meune, Marouane Boubaya, Jérôme Avouac, Eric 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 HesselstrandRadim Becvar, Armando Gabrielli, Nemanja Damjanov, Maria J Salvador, Valeria Riccieri, Carina Mihai, Gabriella Szücs, Ulrich A Walker, Nicolas Hunzelmann, Duska Martinovic, Vanessa Smith, Carolina de Souza Müller, Carlo Maurizio Montecucco, Daniela Opris, Francesca Ingegnoli, Panayiotis G Vlachoyiannopoulos, Bojana Stamenkovic, Edoardo Rosato, Stefan Heitmann, Jörg H W Distler, Thierry Zenone, Matthias Seidel, Alessandra Vacca, Ellen De Langhe, Srdan Novak, Maurizio Cutolo, Luc Mouthon, Jörg Henes, Carlo Chizzolini, Carlos Alberto von Mühlen, EUSTAR group

Dermato- & Venerologisk Afd.

474 Citationer (Scopus)

Abstract

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.

Originalsprog	Engelsk

Vol/bind	76
Antal sider	9
DOI	https://doi.org/10.1136/annrheumdis-2017-211448
Status	Udgivet - nov. 2017

Navn	Annals of the Rheumatic Diseases
ISSN	0003-4967

Adgang til dokumentet

10.1136/annrheumdis-2017-211448

Citationsformater

Elhai, M., Meune, C., Boubaya, M., Avouac, J., Hachulla, E., Balbir-Gurman, A., Riemekasten, G., Airò, P., Joven, B., Vettori, S., Cozzi, F., Ullman, S., Czirják, L., Tikly, M., Müller-Ladner, U., Caramaschi, P., Distler, O., Iannone, F., Ananieva, L. P., ... EUSTAR group (2017). Mapping and predicting mortality from systemic sclerosis. Annals of the Rheumatic Diseases https://doi.org/10.1136/annrheumdis-2017-211448

Elhai, M, Meune, C, Boubaya, M, Avouac, J, Hachulla, E, Balbir-Gurman, A, Riemekasten, G, Airò, P, Joven, B, Vettori, S, Cozzi, F, Ullman, S, Czirják, L, Tikly, M, Müller-Ladner, U, Caramaschi, P, Distler, O, Iannone, F, Ananieva, LP, Hesselstrand, R, Becvar, R, Gabrielli, A, Damjanov, N, Salvador, MJ, Riccieri, V, Mihai, C, Szücs, G, Walker, UA, Hunzelmann, N, Martinovic, D, Smith, V, Müller, CDS, Montecucco, CM, Opris, D, Ingegnoli, F, Vlachoyiannopoulos, PG, Stamenkovic, B, Rosato, E, Heitmann, S, Distler, JHW, Zenone, T, Seidel, M, Vacca, A, Langhe, ED, Novak, S, Cutolo, M, Mouthon, L, Henes, J, Chizzolini, C, Mühlen, CAV & EUSTAR group 2017, Mapping and predicting mortality from systemic sclerosis. Annals of the Rheumatic Diseases, bind 76. https://doi.org/10.1136/annrheumdis-2017-211448

@book{bf9ad181135a475abaef75b3f5a1d892,

title = "Mapping and predicting mortality from systemic sclerosis",

abstract = "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.",

keywords = "Aged, Cause of Death, Databases, Factual, Death Certificates, Female, France, Humans, Male, Middle Aged, Prognosis, Proportional Hazards Models, Risk Factors, Scleroderma, Systemic, Time Factors, Journal Article",

author = "Muriel Elhai and Christophe Meune and Marouane Boubaya and J{\'e}r{\^o}me Avouac and Eric Hachulla and Alexandra Balbir-Gurman and Gabriela Riemekasten and Paolo Air{\`o} and Beatriz Joven and Serena Vettori and Franco Cozzi and Susanne Ullman and L{\'a}szl{\'o} Czirj{\'a}k and Mohammed Tikly and Ulf M{\"u}ller-Ladner and Paola Caramaschi and Oliver Distler and Florenzo Iannone and Ananieva, {Lidia P} and Roger Hesselstrand and Radim Becvar and Armando Gabrielli and Nemanja Damjanov and Salvador, {Maria J} and Valeria Riccieri and Carina Mihai and Gabriella Sz{\"u}cs and Walker, {Ulrich A} and Nicolas Hunzelmann and Duska Martinovic and Vanessa Smith and M{\"u}ller, {Carolina de Souza} and Montecucco, {Carlo Maurizio} and Daniela Opris and Francesca Ingegnoli and Vlachoyiannopoulos, {Panayiotis G} and Bojana Stamenkovic and Edoardo Rosato and Stefan Heitmann and Distler, {J{\"o}rg H W} and Thierry Zenone and Matthias Seidel and Alessandra Vacca and Langhe, {Ellen De} and Srdan Novak and Maurizio Cutolo and Luc Mouthon and J{\"o}rg Henes and Carlo Chizzolini and M{\"u}hlen, {Carlos Alberto von} and {EUSTAR group}",

note = "{\textcopyright} Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.",

year = "2017",

month = nov,

doi = "10.1136/annrheumdis-2017-211448",

language = "English",

volume = "76",

series = "Annals of the Rheumatic Diseases",

publisher = "B M J Group",

}

TY - RPRT

T1 - Mapping and predicting mortality from systemic sclerosis

AU - Elhai, Muriel

AU - Meune, Christophe

AU - Boubaya, Marouane

AU - Avouac, Jérôme

AU - Hachulla, Eric

AU - Balbir-Gurman, Alexandra

AU - Riemekasten, Gabriela

AU - Airò, Paolo

AU - Joven, Beatriz

AU - Vettori, Serena

AU - Cozzi, Franco

AU - Ullman, Susanne

AU - Czirják, László

AU - Tikly, Mohammed

AU - Müller-Ladner, Ulf

AU - Caramaschi, Paola

AU - Distler, Oliver

AU - Iannone, Florenzo

AU - Ananieva, Lidia P

AU - Hesselstrand, Roger

AU - Becvar, Radim

AU - Gabrielli, Armando

AU - Damjanov, Nemanja

AU - Salvador, Maria J

AU - Riccieri, Valeria

AU - Mihai, Carina

AU - Szücs, Gabriella

AU - Walker, Ulrich A

AU - Hunzelmann, Nicolas

AU - Martinovic, Duska

AU - Smith, Vanessa

AU - Müller, Carolina de Souza

AU - Montecucco, Carlo Maurizio

AU - Opris, Daniela

AU - Ingegnoli, Francesca

AU - Vlachoyiannopoulos, Panayiotis G

AU - Stamenkovic, Bojana

AU - Rosato, Edoardo

AU - Heitmann, Stefan

AU - Distler, Jörg H W

AU - Zenone, Thierry

AU - Seidel, Matthias

AU - Vacca, Alessandra

AU - Langhe, Ellen De

AU - Novak, Srdan

AU - Cutolo, Maurizio

AU - Mouthon, Luc

AU - Henes, Jörg

AU - Chizzolini, Carlo

AU - Mühlen, Carlos Alberto von

AU - EUSTAR group

PY - 2017/11

Y1 - 2017/11

N2 - 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.

AB - 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.

KW - Aged

KW - Cause of Death

KW - Databases, Factual

KW - Death Certificates

KW - Female

KW - France

KW - Humans

KW - Male

KW - Middle Aged

KW - Prognosis

KW - Proportional Hazards Models

KW - Risk Factors

KW - Scleroderma, Systemic

KW - Time Factors

KW - Journal Article

U2 - 10.1136/annrheumdis-2017-211448

DO - 10.1136/annrheumdis-2017-211448

M3 - Report

C2 - 28835464

VL - 76

T3 - Annals of the Rheumatic Diseases

BT - Mapping and predicting mortality from systemic sclerosis

ER -

Mapping and predicting mortality from systemic sclerosis

Abstract

Adgang til dokumentet

Fingeraftryk

Citationsformater