Causes and risk factors for death in systemic sclerosis: a study from the EULAR Scleroderma Trials and Research (EUSTAR) database

被引：1088

作者：

Tyndall, Anthony J. ^{[1
]}

Bannert, Bettina ^{[1
]}

Vonk, Madelon ^{[2
]}

Airo, Paolo ^{[3
]}

Cozzi, Franco ^{[4
]}

Carreira, Patricia E. ^{[5
]}

Bancel, Dominique Farge ^{[6
]}

Allanore, Yannick ^{[7
]}

Mueller-Ladner, Ulf ^{[8
,9
]}

Distler, Oliver ^{[10
]}

Iannone, Florenzo ^{[11
]}

Pellerito, Raffaele ^{[12
]}

Pileckyte, Margarita ^{[13
]}

Miniati, Irene ^{[14
]}

Ananieva, Lidia ^{[15
]}

Gurman, Alexandra Balbir ^{[16
]}

Damjanov, Nemanja ^{[17
]}

Mueller, Adelheid ^{[18
]}

Valentini, Gabriele ^{[19
]}

Riemekasten, Gabriela ^{[20
]}

Tikly, Mohammed ^{[21
,22
]}

Hummers, Laura ^{[23
]}

Henriques, Maria J. S. ^{[24
]}

Caramaschi, Paola ^{[25
]}

Scheja, Agneta ^{[26
]}

Rozman, Blaz ^{[27
]}

Ton, Evelien ^{[28
]}

Kumanovics, Gabor ^{[29
]}

Coleiro, Bernard ^{[30
]}

Feierl, Eva ^{[31
]}

Szucs, Gabriella ^{[32
,33
]}

Von Muehlen, Carlos Alberto ^{[34
]}

Riccieri, Valeria ^{[35
]}

Novak, Srdan ^{[36
]}

Chizzolini, Carlo ^{[37
]}

Kotulska, Anna ^{[38
]}

Denton, Christopher ^{[39
]}

Coelho, Paulo C. ^{[40
]}

Koetter, Ina ^{[41
]}

Simsek, Ismail ^{[42
]}

de la Pena Lefebvre, Paloma Garcia ^{[43
]}

Hachulla, Eric ^{[44
]}

Seibold, James R. ^{[45
]}

Rednic, Simona ^{[46
]}

Stork, Jiri ^{[47
]}

Morovic-Vergles, Jadranka ^{[48
]}

Walker, Ulrich A. ^{[1
]}

机构：

[1] Univ Basel, Dept Rheumatol, CH-4012 Basel, Switzerland

[2] Radboud Univ Nijmegen, Med Ctr, NL-6525 ED Nijmegen, Netherlands

[3] Serv Reumatol Allergol & Immunol Clin, Brescia, Italy

[4] Univ Padua, Rheumatol Unit, Dept Clin & Expt Med, Padua, Italy

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

[6] Hop St Louis, Dept Internal Med, Paris, France

[7] Hop Cochin, Dept Rheumatol, F-75674 Paris, France

[8] Max Planck Inst Physiol & Clin Res, Kerckhoff Klin, Abt Rheumatol, D-6350 Bad Nauheim, Germany

[9] Max Planck Inst Physiol & Clin Res, Kerckhoff Klin, Klin Immunol, D-6350 Bad Nauheim, Germany

[10] Univ Zurich Hosp, Dept Rheumatol, CH-8091 Zurich, Switzerland

[11] Univ Bari, UO Reumatol, Bari, Italy

[12] Osped Mauriziano Umberto 1, Ctr Reumatol, Turin, Italy

[13] Kaunas Univ, Hosp Med, Dept Rheumatol, Kaunas, Lithuania

[14] Univ Florence, Div Rheumatol AOUC, Dept Biomed, Florence, Italy

[15] Russian Acad Med Sci, Inst Rheumatol, Moscow 109801, Russia

[16] Rambam Hlth Care Campus, Shine Dept Rheumatol B, Haifa, Israel

[17] Inst Rheumatol, Belgrade, Serbia

[18] Univ Regensburg, Dept Internal Med 1, Regensburg, Germany

[19] Policlin UO Reumatol, Dipartimento Med Clin & Sperimentale F Magrassi 2, Naples, Italy

[20] Charite, Dept Rheumatol, Berlin, Germany

[21] Hosp & Univ Witwatersrand, Johannesburg, South Africa

[22] Dept Med Chris Hani Baragwanath, Rheumatol Unit, Johannesburg, South Africa

[23] Johns Hopkins Univ, Div Rheumatol, Baltimore, MD USA

[24] Hosp Univ Coimbra, Coimbra, Portugal

[25] Univ Verona, Dipartimento Med Clin & Sperimentale, I-37100 Verona, Italy

[26] Univ Lund Hosp, Dept Rheumatol, S-22185 Lund, Sweden

[27] Univ Med Ctr, Dept Rheumatol, Ljubljana, Slovenia

[28] Univ Med Ctr, Utrecht, Netherlands

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

[30] Stella Maris Balzan, Balzan, Malta

[31] Med Univ Vienna, Div Rheumatol, Vienna, Austria

[32] Univ Debrecen, Dept Rheumatol, Inst Internal Med, H-4012 Debrecen, Hungary

[33] Hlth Sci Ctr, Debrecen, Hungary

[34] Rheuma Clin, Porto Alegre, RS, Brazil

[35] Univ Roma La Sapienza, Div Reumatol, Rome, Italy

[36] KBC Rijeka, Dept Rheumatol & Clin Immunol, Rijeka, Croatia

[37] Univ Hosp, Dept Immunol & Allergy, Geneva, Switzerland

[38] Med Univ Silesia, Dept Internal Med & Rheumatol, Katowice, Poland

[39] Royal Free & Univ Coll London Med Sch, Ctr Rheumatol, London, England

[40] Inst Portugues Reumatol, Lisbon, Portugal

[41] Univ Klinikum Tubingen, Tubingen, Germany

[42] Gulhane Mil Med Acad, Div Rheumatol, Ankara, Turkey

[43] Hosp Ramon & Cajal, Serv Reumatol, E-28034 Madrid, Spain

[44] Hop Claude Huriez, Dept Internal Med, Lille, France

[45] Univ Michigan, Scleroderma Program, Ann Arbor, MI 48109 USA

[46] Univ Med & Pharm Iuliu Hatieganu, Clin Reumatol, Cluj Napoca, Romania

[47] Charles Univ Prague, Dept Dermatol, Prague, Czech Republic

[48] Dubrava Univ Hosp, Div Clin Immunol & Rheumatol, Zagreb, Croatia

来源：

ANNALS OF THE RHEUMATIC DISEASES | 2010年 / 69卷 / 10期

关键词：

SURVIVAL; MORTALITY; DISEASE; ATHEROSCLEROSIS; MANIFESTATIONS; HYPERTENSION; ACCURACY; SPECTRUM; FIBROSIS;

D O I：

10.1136/ard.2009.114264

中图分类号：

R5 [内科学];

学科分类号：

1002 ; 100201 ;

摘要：

Objectives To determine the causes and predictors of mortality in systemic sclerosis (SSc). Methods Patients with SSc (n=5860) fulfilling the American College of Rheumatology criteria and prospectively followed in the EULAR Scleroderma Trials and Research (EUSTAR) cohort were analysed. EUSTAR centres completed a structured questionnaire on cause of death and comorbidities. Kaplan-Meier and Cox proportional hazards models were used to analyse survival in SSc subgroups and to identify predictors of mortality. Results Questionnaires were obtained on 234 of 284 fatalities. 55% of deaths were attributed directly to SSc and 41% to non-SSc causes; in 4% the cause of death was not assigned. Of the SSc-related deaths, 35% were attributed to pulmonary fibrosis, 26% to pulmonary arterial hypertension (PAH) and 26% to cardiac causes (mainly heart failure and arrhythmias). Among the non-SSc-related causes, infections (33%) and malignancies (31%) were followed by cardiovascular causes (29%). Of the non-SSc-related fatalities, 25% died of causes in which SSc-related complications may have participated (pneumonia, sepsis and gastrointestinal haemorrhage). Independent risk factors for mortality and their HR were: proteinuria (HR 3.34), the presence of PAH based on echocardiography (HR 2.02), pulmonary restriction (forced vital capacity below 80% of normal, HR 1.64), dyspnoea above New York Heart Association class II (HR 1.61), diffusing capacity of the lung (HR 1.20 per 10% decrease), patient age at onset of Raynaud's phenomenon (HR 1.30 per 10 years) and the modified Rodnan skin score (HR 1.20 per 10 score points). Conclusion Disease-related causes, in particular pulmonary fibrosis, PAH and cardiac causes, accounted for the majority of deaths in SSc.

引用

页码：1809 / 1815

页数：7

共 31 条

[1] Angiographically proven coronary artery disease in scleroderma [J].

Akram, M. R. ;

Handler, C. E. ;

Williams, M. ;

Carulli, M. T. ;

Andron, M. ;

Black, C. M. ;

Denton, C. P. ;

Coghlan, J. G. .

RHEUMATOLOGY, 2006, 45 (11) :1395-1398

[2] Prevalence and factors associated with left ventricular dysfunction in the EULAR Scleroderma Trial and Research group (EUSTAR) database of patients with systemic sclerosis [J].

Allanore, Y. ;

Meune, C. ;

Vonk, M. C. ;

Airo, P. ;

Hachulla, E. ;

Caramaschi, P. ;

Riemekasten, G. ;

Cozzi, F. ;

Beretta, L. ;

Derk, C. T. ;

Komocsi, A. ;

Farge, D. ;

Balbir, A. ;

Riccieri, V. ;

Distler, O. ;

Chiala, A. ;

Del Papa, N. ;

Simic, K. Pasalic ;

Ghio, M. ;

Stamenkovic, B. ;

Rednic, S. ;

Host, N. ;

Pellerito, R. ;

Zegers, E. ;

Kahan, A. ;

Walker, U. A. ;

Matucci-Cerinic, M. .

ANNALS OF THE RHEUMATIC DISEASES, 2010, 69 (01) :218-221

[3] PREDICTORS OF SURVIVAL IN SYSTEMIC-SCLEROSIS (SCLERODERMA) [J].

ALTMAN, RD ;

MEDSGER, TA ;

BLOCH, DA ;

MICHEL, BA .

ARTHRITIS AND RHEUMATISM, 1991, 34 (04) :403-413

[4] PRELIMINARY CRITERIA FOR THE CLASSIFICATION OF SYSTEMIC-SCLEROSIS (SCLERODERMA) [J].

不详 .

ARTHRITIS AND RHEUMATISM, 1980, 23 (05) :581-590

[5]

Bryan C, 1999, ARTHRITIS RHEUM-US, V42, P2660, DOI 10.1002/1529-0131(199912)42:12<2660::AID-ANR23>3.0.CO

[6]

2-N

[7] Risk of malignancy in scleroderma - A population-based cohort study [J].

Chatterjee, S ;

Dombi, GW ;

Severson, RK ;

Mayes, MD .

ARTHRITIS AND RHEUMATISM, 2005, 52 (08) :2415-2424

[8] Hospitalizations and mortality in systemic sclerosis: results from the Nationwide Inpatient Sample [J].

Chung, L. ;

Krishnan, E. ;

Chakravarty, E. F. .

RHEUMATOLOGY, 2007, 46 (12) :1808-1813

[9] Survival and causes of death in 366 Hungarian patients with systemic sclerosis [J].

Czirjak, L. ;

Kumanovics, G. ;

Varju, C. ;

Nagy, Z. ;

Pakozdi, A. ;

Szekanecz, Z. ;

Szucs, G. .

ANNALS OF THE RHEUMATIC DISEASES, 2008, 67 (01) :59-63

[10] Autopsy findings in 42 consecutive patients with idiopathic pulmonary fibrosis [J].

Daniels, C. E. ;

Yi, E. S. ;

Ryu, J. H. .

EUROPEAN RESPIRATORY JOURNAL, 2008, 32 (01) :170-174

← 1 2 3 4 →