Spanish scleroderma risk score (RESCLESCORE) to predict 15-year all-cause mortality in scleroderma patients at the time of diagnosis based on the RESCLE cohort: Derivation and internal validation

被引：15

作者：

Rubio-Rivas, Manuel ^{[1
]}

Corbella, Xavier ^{[1
,2
]}

Guillen-del-Castillo, Alfredo ^{[3
]}

Tolosa Vilella, Carles ^{[4
]}

Colunga Arguelles, Dolores ^{[5
]}

Argibay, Ana ^{[6
]}

Vargas Hitos, Jose Antonio ^{[7
]}

Todoli Parra, Jose Antonio ^{[8
]}

Gonzalez-Echavarri, Cristina ^{[9
]}

Ortego-Centeno, Norberto ^{[10
]}

Trapiella Martinez, Luis ^{[11
]}

Rodriguez Carballeira, Monica ^{[12
]}

Marin Ballve, Adela ^{[13
]}

Pla Salas, Xavier ^{[14
]}

Perales Fraile, Isabel ^{[15
]}

Chamorro, Antonio-J ^{[16
]}

Madronero Vuelta, Ana Belen ^{[17
]}

Freire, Mayka ^{[18
]}

Ruiz Munoz, Manuel ^{[19
]}

Gonzalez Garcia, Andres ^{[20
]}

Martin del Campo, Isaac Pons ^{[21
]}

Sanchez Garcia, Maria Esther ^{[22
]}

Bernal Bello, David ^{[23
]}

Espinosa, Gerard ^{[24
]}

Garcia Hernandez, Francisco Jose ^{[25
]}

Saez Comet, Luis ^{[26
]}

Rios Blanco, Juan Jose ^{[27
]}

de la Puebla Gimenez, Rafael Angel Fernandez ^{[28
]}

Sanchez Trigo, Sabela ^{[29
]}

Fonollosa Pla, Vicent ^{[3
]}

Simeon Aznar, Carmen Pilar ^{[3
]}

机构：

[1] Bellvitge Univ Hosp IDIBELL, Dept Internal Med, Autoimmune Dis Unit, Barcelona, Spain

[2] Univ Int Catalunya, Fac Med & Hlth Sci, Hestia Chair Integrated Hlth & Social Care, Grp Evaluat Hlth Determinants & Hlth Policies, Barcelona, Spain

[3] Hosp Univ Vall dHebron, Dept Internal Med, Unit Autoimmune Dis, Barcelona, Spain

[4] Corp Sanitaria Univ Parc Tauli, Dept Internal Med, Barcelona, Spain

[5] Hosp Univ Cent Asturias, Dept Internal Med, Oviedo, Asturias, Spain

[6] Complejo Hosp Univ Vigo, Dept Internal Med, Unit Syst Autoimmune Dis & Thrombosis, Vigo, Pontevedra, Spain

[7] Hosp Univ Virgen Nieves, Dept Internal Med, Granada, Spain

[8] Hosp Univ & Politecn La Fe, Dept Internal Med, Valencia, Spain

[9] Univ Basque Country, Hosp Univ Cruces, Biocruces Bizkaia Hlth Res Inst, Autoimmune Dis Res Unit,Dept Internal Med, Baracaldo, Spain

[10] Hosp Univ San Cecilio, Fac Med, Inst Invest Biosanitaria Ibs Granada, Dept Internal Med,Unit Syst Autoimmune Dis, Granada, Spain

[11] Hosp Cabuenes, Dept Internal Med, Unit Syst Autoimmune Dis, Gijon, Asturias, Spain

[12] Hosp Univ Mutua Terrassa, Dept Internal Med, Barcelona, Spain

[13] Hosp Clin Univ Lozano Blesa, Dept Internal Med, Unit Autoimmune Dis, Zaragoza, Spain

[14] Consorci Hosp Vic, Dept Internal Med, Unit Syst Autoimmune Dis, Barcelona, Spain

[15] Hosp Univ Rey Juan Carlos, Dept Internal Med, Madrid, Spain

[16] Complejo Asistencial Univ Salamanca, Dept Internal Med, Salamanca, Spain

[17] Hosp Gen San Jorge, Dept Internal Med, Huesca, Spain

[18] Hosp Clin Univ Santiago, Dept Internal Med, Santiago De Compostela, A Coruna, Spain

[19] Hosp Univ Fdn Alcorcon, Dept Internal Med, Madrid, Spain

[20] Hosp Univ Ramon y Cajal, Dept Internal Med, Madrid, Spain

[21] Xarxa Assistencial Univ Manresa, Dept Internal Med, Barcelona, Spain

[22] Hosp Univ Virgen Valme, Dept Internal Med, Seville, Spain

[23] Hosp Univ Fuenlabrada, Dept Internal Med, Madrid, Spain

[24] Hosp Clin Barcelona, Dept Autoimmune Dis, Barcelona, Spain

[25] Hosp Univ Virgen Rocio, Dept Internal Med, Seville, Spain

[26] Hosp Univ Miguel Servet, Dept Internal Med, Zaragoza, Spain

[27] Hosp Univ La Paz, Dept Internal Med, Madrid, Spain

[28] Hosp Univ Reina Sofia, Dept Internal Med, Cordoba, Spain

[29] Complejo Hosp Univ Ferrol, Dept Internal Med, Ferrol, A Coruna, Spain

来源：

AUTOIMMUNITY REVIEWS | 2020年 / 19卷 / 05期

关键词：

Systemic sclerosis; Prognosis; Mortality; Score; SYSTEMIC-SCLEROSIS PATIENTS; 5-YEAR MORTALITY; SURVIVAL; DISEASE; DEATH; MODEL; CLASSIFICATION; FEATURES; CRITERIA; RULE;

D O I：

10.1016/j.autrev.2020.102507

中图分类号：

R392 [医学免疫学]; Q939.91 [免疫学];

学科分类号：

100102 ;

摘要：

A few scores predicting the short-term risk of mortality in Systemic sclerosis (SSc) have been reported to date. Our study aimed to create a predictive 15-year all-cause mortality score at the time of the diagnosis of SSc. The study was based on the Spanish Scleroderma Registry (RESCLE). The cohort was split up in derivation (DC) and validation cohort (VC). A multivariate analysis to detect variables related to all-cause mortality within the first 15 years from SSc diagnosis was performed, assigning points to the rounded beta values to create the score (RESCLESCORE). 1935 SSc patients were included. The variables in the final model were as follows: age at diagnosis (+2 points > 65 years-old), male gender (+1 point), lcSSc subset (-1 point), mode of onset other than Raynaud's (+1 point), cancer (+1 point) and visceral involvement, such as ILD (+1 point), PAH (+1 point), heart ( +1 point) and renal involvement ( +2 points). Autoantibodies did not achieve statistical significance in the multivariate analysis. The 3 categories of risk to predict 15-year all-cause mortality at the time of diagnosis were as follows: low risk (5% vs. 7%, p = .189), intermediate risk (26.5% vs. 25.5%, p = .911) and high risk (47.8% vs. 59%, p = .316). The AUC was 0.799 (DC) vs. 0.778 (VC) (p = .530). In conclusion, the RESCLESCORE demonstrated an excellent ability to categorize SSc patients at the time of diagnosis in separate 15-year all-cause mortality risk strata at the time of diagnosis.

引用

页数：7

共 37 条

[1] Early- versus Late-Onset Systemic Sclerosis Differences in Clinical Presentation and Outcome in 1037 Patients [J].

Alba, Marco A. ;

Velasco, Cesar ;

Pilar Simeon, Carmen ;

Fonollosa, Vicent ;

Trapiella, Luis ;

Victoria Egurbide, Maria ;

Saez, Luis ;

Jesus Castillo, Maria ;

Luis Callejas, Jose ;

Teresa Camps, Maria ;

Tolosa, Carles ;

Jose Rios, Juan ;

Freire, Mayka ;

Antonio Vargas, Jose ;

Espinosa, Gerard .

MEDICINE, 2014, 93 (02) :73-81

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

不详 .

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

[3] Clinical and Genetic Factors Predictive of Mortality in Early Systemic Sclerosis [J].

Assassi, Shervin ;

del Junco, Deborah ;

Sutter, Kari ;

McNearney, Terry A. ;

Reveille, John D. ;

Karnavas, Andrew ;

Gourh, Pravitt ;

Estrada-Y-Martin, Rosa M. ;

Fischbach, Michael ;

Arnett, Frank C. ;

Mayes, Maureen D. .

ARTHRITIS CARE & RESEARCH, 2009, 61 (10) :1403-1411

[4]

Beretta L, 2010, CLIN EXP RHEUMATOL, V28, pS18

[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] 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

[8] Derivation and External Validation of a Prediction Rule for Five-Year Mortality in Patients With Early Diffuse Cutaneous Systemic Sclerosis [J].

Domsic, Robyn T. ;

Nihtyanova, Svetlana I. ;

Wisniewski, Stephen R. ;

Fine, Michael J. ;

Lucas, Mary ;

Kwoh, C. Kent ;

Denton, Christopher P. ;

Medsger, Thomas A., Jr. .

ARTHRITIS & RHEUMATOLOGY, 2016, 68 (04) :993-1003

[9] Derivation and Validation of a Prediction Rule for Two-Year Mortality in Early Diffuse Cutaneous Systemic Sclerosis [J].

Domsic, Robyn T. ;

Nihtyanova, Svetlana I. ;

Wisniewski, Stephen R. ;

Fine, Michael J. ;

Lucas, Mary ;

Kwoh, C. Kent ;

Denton, Christopher P. ;

Medsger, Thomas A., Jr. .

ARTHRITIS & RHEUMATOLOGY, 2014, 66 (06) :1616-1624

[10] Mapping and predicting mortality from systemic sclerosis [J].

Elhai, Muriel ;

Meune, Christophe ;

Boubaya, Marouane ;

Avouac, Jerome ;

Hachulla, Eric ;

Balbir-Gurman, Alexandra ;

Riemekasten, Gabriela ;

Airo, Paolo ;

Joven, Beatriz ;

Vettori, Serena ;

Cozzi, Franco ;

Ullman, Susanne ;

Czirjak, Laszlo ;

Tikly, Mohammed ;

Mueller-Ladner, U. L. F. ;

Caramaschi, Paola ;

Distler, Oliver ;

Iannone, Florenzo ;

Ananieva, Lidia P. ;

Hesselstrand, Roger ;

Becvar, Radim ;

Gabrielli, Armando ;

Damjanov, Nemanja ;

Salvador, Maria J. ;

Riccieri, Valeria ;

Mihai, Carina ;

Szucs, Gabriella ;

Walker, Ulrich A. ;

Hunzelmann, Nicolas ;

Martinovic, Duska ;

Smith, Vanessa ;

Mueller, Carolina de Souza ;

Montecucco, Carlo Maurizio ;

Opris, Daniela ;

Ingegnoli, Francesca ;

Vlachoyiannopoulos, Panayiotis G. ;

Stamenkovic, Bojana ;

Rosato, Edoardo ;

Heitmann, Stefan ;

Distler, Joerg H. W. ;

Zenone, Thierry ;

Seidel, Matthias ;

Vacca, Alessandra ;

De langhe, Ellen ;

Novak, Srdan ;

Cutolo, Maurizio ;

Mouthon, Luc ;

Henes, Joerg ;

Chizzolini, Carlo ;

von Muhlen, Carlos Alberto .

ANNALS OF THE RHEUMATIC DISEASES, 2017, 76 (11) :1897-1905

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