Mapping and predicting mortality from systemic sclerosis

被引：547

作者：

Elhai, Muriel ^{[1
]}

Meune, Christophe ^{[2
]}

Boubaya, Marouane ^{[3
]}

Avouac, Jerome ^{[1
]}

Hachulla, Eric ^{[4
]}

Balbir-Gurman, Alexandra ^{[5
]}

Riemekasten, Gabriela ^{[6
]}

Airo, Paolo ^{[7
]}

Joven, Beatriz ^{[8
]}

Vettori, Serena ^{[9
]}

Cozzi, Franco ^{[10
]}

Ullman, Susanne ^{[11
]}

Czirjak, Laszlo ^{[12
]}

Tikly, Mohammed ^{[13
]}

Mueller-Ladner, U. L. F. ^{[14
]}

Caramaschi, Paola ^{[15
]}

Distler, Oliver ^{[16
]}

Iannone, Florenzo ^{[17
]}

Ananieva, Lidia P. ^{[18
]}

Hesselstrand, Roger ^{[19
]}

Becvar, Radim ^{[20
]}

Gabrielli, Armando ^{[21
]}

Damjanov, Nemanja ^{[22
]}

Salvador, Maria J. ^{[23
]}

Riccieri, Valeria ^{[24
]}

Mihai, Carina ^{[25
,26
]}

Szucs, Gabriella ^{[27
]}

Walker, Ulrich A. ^{[28
]}

Hunzelmann, Nicolas ^{[29
]}

Martinovic, Duska ^{[30
]}

Smith, Vanessa ^{[31
]}

Mueller, Carolina de Souza ^{[32
]}

Montecucco, Carlo Maurizio ^{[33
]}

Opris, Daniela ^{[34
]}

Ingegnoli, Francesca ^{[35
]}

Vlachoyiannopoulos, Panayiotis G. ^{[36
]}

Stamenkovic, Bojana ^{[37
]}

Rosato, Edoardo ^{[38
]}

Heitmann, Stefan ^{[39
]}

Distler, Joerg H. W. ^{[40
]}

Zenone, Thierry ^{[41
]}

Seidel, Matthias ^{[42
]}

Vacca, Alessandra ^{[43
]}

De langhe, Ellen ^{[44
,45
,46
]}

Novak, Srdan ^{[47
]}

Cutolo, Maurizio ^{[48
,49
]}

Mouthon, Luc ^{[50
]}

Henes, Joerg ^{[51
]}

Chizzolini, Carlo ^{[52
]}

von Muhlen, Carlos Alberto ^{[53
]}

机构：

[1] Paris Descartes Univ, Rheumatol Dept A, INSERM, U1016,Sorbonne Paris Cite,Cochin Hosp, Paris, France

[2] Paris XIII Univ, Bobigny Hosp, Dept Cardiol, INSERM,UMR S 942, Paris, France

[3] Paris Seine St Denis Univ, Unit Clin Res, Bobigny, France

[4] Univ Lille Nord de France, Hop Claude Huriez, Dept Internal Med, Lille, France

[5] Technion Israel Inst Technol, Rappaport Fac Med, Rambam Hlth Care Campus, Shine Rheumatol Unit B, Haifa, Israel

[6] Univ Lubeck, Dept Rheumatol, Lubeck, Germany

[7] Spedali Civili Brescia, UO Reumatol & Immunol Clin, Brescia, Italy

[8] Hosp Univ 12 Octubre, Serv Reumatol, Madrid, Spain

[9] F Magrassi II, Dept Clin & Expt Med, Naples, Italy

[10] Univ Padua, Dept Med, Rheumatol Unit, Padua, Italy

[11] Hosp Bispebjerg, Univ Hosp Copenhagen, Dept Dermatol, Copenhagen, Denmark

[12] Univ Pecs, Dept Immunol & Rheumatol, Pecs, Hungary

[13] Univ Witwatersrand, Chris Hani Baragwanath Acad Hosp, Johannesburg, South Africa

[14] Justus Liebig Univ Giessen, Dept Rheumatol & Clin Immunol, Kerckhoff Clin, Bad Nauheim, Germany

[15] Univ Verona, Dept Med, Rheumatol Unit, Verona, Italy

[16] Univ Hosp Zurich, Dept Rheumatol, Zurich, Switzerland

[17] Univ Bari, Interdisciplinary Dept Med Rheumatol Unit, Bari, Italy

[18] VA Nasonova Inst Rheumatol, Moscow, Russia

[19] Lund Univ, Skane Univ Hosp, Rheumatol Sect, Dept Clin Sci Lund, Lund, Sweden

[20] Charles Univ Prague, Med Sch 1, Inst Rheumatol, Prague, Czech Republic

[21] Univ Politecn Marche, Dipartimento Sci Clin & Mol, Clin Med, Ancona, Italy

[22] Univ Belgrade, Sch Med, Inst Rheumatol, Belgrade, Serbia

[23] Hosp Univ, Rheumatol Dept, Coimbra, Portugal

[24] Sapienza Univ Rome, Dept Internal Med & Med Special, Rome, Italy

[25] Carol Davila Univ Med & Pharm, Ion Cantacuzino Clin Hosp, Dept Internal Med, Bucharest, Romania

[26] Carol Davila Univ Med & Pharm, Ion Cantacuzino Clin Hosp, Rheumatol Clin, Bucharest, Romania

[27] Univ Debrecen, Div Rheumatol, Dept Internal Med, Debrecen, Hungary

[28] Basel Univ, Dept Rheumatol, Unispital Basel, Basel, Switzerland

[29] Univ Hosp Cologne, Dept Dermatol, Cologne, Germany

[30] Clin Hosp Split, Dept Internal Med, Split, Croatia

[31] Univ Ghent, Dept Rheumatol, Ghent, Belgium

[32] Univ Fed Parana, Clin Hosp, Rheumatol Div, Curitiba, Parana, Brazil

[33] IRCCS Policlin S Matteo, Unita Operat & Cattedra Reumatol, Pavia, Italy

[34] Carol Davila Univ Med & Pharm, St Maria Hosp, Dept Rheumatol, Bucharest, Romania

[35] Univ Milan, Ist Ortoped Gaetano Pini, Dipartimento & Cattedra Reumatol, Milan, Italy

[36] Natl Univ & Kapodistrian Athens, Sch Med, Dept Pathophysiol, Athens, Greece

[37] Inst Prevent Treatment & Rehabil Rheumat & Cardio, Niska Banja, Serbia

[38] Univ Roma La Sapienza, Policlin Umberto 1, Ctr Sclerosi Sistem, Dipartimento Med Clin, Rome, Italy

[39] Marienhosp Stuttgart, Dept Rheumatol, Stuttgart, Germany

[40] Univ Hosp Erlangen, Dept Internal Med 3, Erlangen, Germany

[41] Dept Med, Unit Internal Med, Valence, France

[42] Univ Hosp Bonn, Med Klin 3, Bonn, Germany

[43] Univ Hosp Cagliari, Rheumatol Unit, Monserrato, Italy

[44] Univ Hosp Leuven, Div Rheumatol, Leuven, Belgium

[45] Univ Hosp Leuven, Dept Dev & Regenerat, Leuven, Belgium

[46] Lab Tissue Homeostasis & Dis, Leuven, Belgium

[47] KBC Rijeka, Dept Rheumatol & Clin Immunol, Internal Med, Rijeka, Croatia

[48] Univ Genoa, Res Lab, Genoa, Italy

[49] Univ Genoa, Div Rheumatol, Dept Internal Med, Genoa, Italy

[50] Hop Cochin, Dept Internal Med, Paris, France

来源：

ANNALS OF THE RHEUMATIC DISEASES | 2017年 / 76卷 / 11期

关键词：

EULAR SCLERODERMA TRIALS; EUSTAR DATABASE; 5-YEAR SURVIVAL; COHORT; MODEL; METAANALYSIS; DEATH; POPULATION; VALIDATION; PROFILE;

D O I：

10.1136/annrheumdis-2017-211448

中图分类号：

R5 [内科学];

学科分类号：

100201 [内科学];

摘要：

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.

引用

页码：1897 / 1905

页数：9

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