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Mapping and predicting mortality from systemic sclerosis
M. Elhai, C. Meune, M. Boubaya, J. Avouac, E. Hachulla, A. Balbir-Gurman, G. Riemekasten, P. Airò, B. Joven, S. Vettori, F. Cozzi, S. Ullman, L. Czirják, M. Tikly, U. Müller-Ladner, P. Caramaschi, O. Distler, F. Iannone, LP. Ananieva, R....
Language English Country Great Britain
Document type Journal Article
NLK
ProQuest Central
from 1939-01-01 to 2024-12-31
Health & Medicine (ProQuest)
from 1939-01-01 to 2024-12-31
Family Health Database (ProQuest)
from 1939-01-01 to 2024-12-31
ROAD: Directory of Open Access Scholarly Resources
- MeSH
- Time Factors MeSH
- Databases, Factual MeSH
- Middle Aged MeSH
- Humans MeSH
- Cause of Death MeSH
- Prognosis MeSH
- Proportional Hazards Models MeSH
- Risk Factors MeSH
- Aged MeSH
- Scleroderma, Systemic mortality MeSH
- Death Certificates MeSH
- Check Tag
- Middle Aged MeSH
- Humans MeSH
- Male MeSH
- Aged MeSH
- Female MeSH
- Publication type
- Journal Article MeSH
- Geographicals
- France MeSH
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.
Chris Hani Baragwanath Academic Hospital University of the Witwatersrand Johannesburg South Africa
Department of Cardiology Paris XIII University INSERM UMR S 942 Bobigny Hospital Paris France
Department of Clinical and Experimental Medicine 'F Magrassi' 2 Naples Italy
Department of Dermatology and Allergy of the TU Munich Munich Germany
Department of Dermatology University Hospital Cologne Cologne Germany
Department of Dermatology University Hospital of Copenhagen Hospital Bispebjerg Copenhagen Denmark
Department of Immunology and Allergy University Hospital Geneva Switzerland
Department of Immunology and Rheumatology University of Pécs Pécs Hungary
Department of Internal Medicine 3 University Hospital Erlangen Erlangen Germany
Department of Internal Medicine Clinical Hospital of Split Split Croatia
Department of Internal Medicine Division of Rheumatology University of Debrecen Debrecen Hungary
Department of Internal Medicine Hôpital Cochin Paris France
Department of Medicine Unit of Internal Medicine Valence cedex France
Department of Medicine University of Otago Christchurch New Zealand
Department of Rheumatology and Clinical Immunology Internal Medicine KBC Rijeka Rijeka Croatia
Department of Rheumatology and Clinical Immunology James Cook University Hospital Middlesbrough UK
Department of Rheumatology and Internal Medicine Medical University of Bialystok Bialystok Poland
Department of Rheumatology Basel University Unispital Basel Basel Switzerland
Department of Rheumatology Hospital Universitario Dr Peset Valencia Spain
Department of Rheumatology Marienhospital Stuttgart Stuttgart Germany
Department of Rheumatology University Hospital Zurich Zurich Switzerland
Department of Rheumatology University of Ghent Ghent Belgium
Department of Rheumatology University of Lübeck Lübeck Germany
Department of Rheumatology University of Marmara Istanbul Turkey
INSERM CépiDc Le Kremlin Bicêtre Le Kremlin Bicêtre France
Institute of Rheumatology 1st Medical School Charles University Praha Czech Republic
Institute of Rheumatology University of Belgrade Medical School Belgrade Serbia
Interdisciplinary Department of Medicine Rheumatology Unit Policlinico University of Bari Bari Italy
Medizinische Klinik 3 University Hospital of Bonn Bonn Germany
Medizinische Universitätsklinik Abt 2 Tübingen Germany
Rheuma Clinic Av Carlos Gomes Porto Alegre Porto Alegre Brazil
Rheumatology Department Hospitais da Universidade Coimbra Portugal
Rheumatology Department Hospital Universitario Madrid Norte Sanchinarro Madrid Spain
Rheumatology Division Clinics Hospital Federal University of Paraná Curitiba Brazil
Rheumatology Granollers General Hospital Barcelona Spain
Rheumatology Unit Department of Medicine University of Padova Padova Italy
Rheumatology Unit Department of Medicine University of Verona Verona Italy
Rheumatology Unit University Hospital of Cagliari Monserrato Italy
Rheumatology Unit Waikato University Hospital Hamilton City Hamilton New Zealand
Servicio de Reumatología Hospital Ramon Y Cajal Madrid Spain
Servicio de Reumatologia Hospital Universitario 12 de Octubre Madrid Spain
Unit of Clinical Research Paris Seine Saint Denis University Bobigny France
Unita Operativa e Cattedra di Reumatologia IRCCS Policlinico S Matteo Pavia Italy
UO Reumatologia ed Immunologia Clinica Spedali Civili Brescia Brescia Italy
VA Nasonova Institute of Rheumatology Moscow Russian Federation
References provided by Crossref.org
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- $a Mapping and predicting mortality from systemic sclerosis / $c M. Elhai, C. Meune, M. Boubaya, J. Avouac, E. Hachulla, A. Balbir-Gurman, G. Riemekasten, P. Airò, B. Joven, S. Vettori, F. Cozzi, S. Ullman, L. Czirják, M. Tikly, U. Müller-Ladner, P. Caramaschi, O. Distler, F. Iannone, LP. Ananieva, R. Hesselstrand, R. Becvar, A. Gabrielli, N. Damjanov, MJ. Salvador, V. Riccieri, C. Mihai, G. Szücs, UA. Walker, N. Hunzelmann, D. Martinovic, V. Smith, CS. Müller, CM. Montecucco, D. Opris, F. Ingegnoli, PG. Vlachoyiannopoulos, B. Stamenkovic, E. Rosato, S. Heitmann, JHW. Distler, T. Zenone, M. Seidel, A. Vacca, E. Langhe, S. Novak, M. Cutolo, L. Mouthon, J. Henes, C. Chizzolini, CAV. Mühlen, K. Solanki, S. Rednic, L. Stamp, B. Anic, VO. Santamaria, M. De Santis, S. Yavuz, WA. Sifuentes-Giraldo, E. Chatelus, J. Stork, JV. Laar, E. Loyo, P. García de la Peña Lefebvre, K. Eyerich, V. Cosentino, JJ. Alegre-Sancho, O. Kowal-Bielecka, G. Rey, M. Matucci-Cerinic, Y. Allanore, . ,
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- $a 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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