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Mortality risk factors in primary Sjögren syndrome: a real-world, retrospective, cohort study

BACKGROUND: What baseline predictors would be involved in mortality in people with primary Sjögren syndrome (SjS) remains uncertain. This study aimed to investigate the baseline characteristics collected at the time of diagnosis of SjS associated with mortality and to identify mortality risk factors...

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Autores principales: Brito-Zerón, Pilar, Flores-Chávez, Alejandra, Horváth, Ildiko Fanny, Rasmussen, Astrid, Li, Xiaomei, Olsson, Peter, Vissink, Arjan, Priori, Roberta, Armagan, Berkan, Hernandez-Molina, Gabriela, Praprotnik, Sonja, Quartuccio, Luca, Inanç, Nevsun, Özkızıltaş, Burcugül, Bartoloni, Elena, Sebastian, Agata, Romão, Vasco C., Solans, Roser, Pasoto, Sandra G., Rischmueller, Maureen, Galisteo, Carlos, Suzuki, Yasunori, Trevisani, Virginia Fernandes Moça, Fugmann, Cecilia, González-García, Andrés, Carubbi, Francesco, Jurcut, Ciprian, Shimizu, Toshimasa, Retamozo, Soledad, Atzeni, Fabiola, Hofauer, Benedikt, Melchor-Díaz, Sheila, Gheita, Tamer, López-Dupla, Miguel, Fonseca-Aizpuru, Eva, Giacomelli, Roberto, Vázquez, Marcos, Consani, Sandra, Akasbi, Miriam, Nakamura, Hideki, Szántó, Antónia, Farris, A. Darise, Wang, Li, Mandl, Thomas, Gattamelata, Angelica, Kilic, Levent, Pirkmajer, Katja Perdan, Abacar, Kerem, Tufan, Abdurrahman, de Vita, Salvatore, Bootsma, Hendrika, Ramos-Casals, Manuel
Formato: Online Artículo Texto
Lenguaje:English
Publicado: Elsevier 2023
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10344811/
https://www.ncbi.nlm.nih.gov/pubmed/37457113
http://dx.doi.org/10.1016/j.eclinm.2023.102062
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author Brito-Zerón, Pilar
Flores-Chávez, Alejandra
Horváth, Ildiko Fanny
Rasmussen, Astrid
Li, Xiaomei
Olsson, Peter
Vissink, Arjan
Priori, Roberta
Armagan, Berkan
Hernandez-Molina, Gabriela
Praprotnik, Sonja
Quartuccio, Luca
Inanç, Nevsun
Özkızıltaş, Burcugül
Bartoloni, Elena
Sebastian, Agata
Romão, Vasco C.
Solans, Roser
Pasoto, Sandra G.
Rischmueller, Maureen
Galisteo, Carlos
Suzuki, Yasunori
Trevisani, Virginia Fernandes Moça
Fugmann, Cecilia
González-García, Andrés
Carubbi, Francesco
Jurcut, Ciprian
Shimizu, Toshimasa
Retamozo, Soledad
Atzeni, Fabiola
Hofauer, Benedikt
Melchor-Díaz, Sheila
Gheita, Tamer
López-Dupla, Miguel
Fonseca-Aizpuru, Eva
Giacomelli, Roberto
Vázquez, Marcos
Consani, Sandra
Akasbi, Miriam
Nakamura, Hideki
Szántó, Antónia
Farris, A. Darise
Wang, Li
Mandl, Thomas
Gattamelata, Angelica
Kilic, Levent
Pirkmajer, Katja Perdan
Abacar, Kerem
Tufan, Abdurrahman
de Vita, Salvatore
Bootsma, Hendrika
Ramos-Casals, Manuel
author_facet Brito-Zerón, Pilar
Flores-Chávez, Alejandra
Horváth, Ildiko Fanny
Rasmussen, Astrid
Li, Xiaomei
Olsson, Peter
Vissink, Arjan
Priori, Roberta
Armagan, Berkan
Hernandez-Molina, Gabriela
Praprotnik, Sonja
Quartuccio, Luca
Inanç, Nevsun
Özkızıltaş, Burcugül
Bartoloni, Elena
Sebastian, Agata
Romão, Vasco C.
Solans, Roser
Pasoto, Sandra G.
Rischmueller, Maureen
Galisteo, Carlos
Suzuki, Yasunori
Trevisani, Virginia Fernandes Moça
Fugmann, Cecilia
González-García, Andrés
Carubbi, Francesco
Jurcut, Ciprian
Shimizu, Toshimasa
Retamozo, Soledad
Atzeni, Fabiola
Hofauer, Benedikt
Melchor-Díaz, Sheila
Gheita, Tamer
López-Dupla, Miguel
Fonseca-Aizpuru, Eva
Giacomelli, Roberto
Vázquez, Marcos
Consani, Sandra
Akasbi, Miriam
Nakamura, Hideki
Szántó, Antónia
Farris, A. Darise
Wang, Li
Mandl, Thomas
Gattamelata, Angelica
Kilic, Levent
Pirkmajer, Katja Perdan
Abacar, Kerem
Tufan, Abdurrahman
de Vita, Salvatore
Bootsma, Hendrika
Ramos-Casals, Manuel
author_sort Brito-Zerón, Pilar
collection PubMed
description BACKGROUND: What baseline predictors would be involved in mortality in people with primary Sjögren syndrome (SjS) remains uncertain. This study aimed to investigate the baseline characteristics collected at the time of diagnosis of SjS associated with mortality and to identify mortality risk factors for all-cause death and deaths related to systemic SjS activity measured by the ESSDAI score. METHODS: In this international, real-world, retrospective, cohort study, we retrospectively collected data from 27 countries on mortality and causes of death from the Big Data Sjögren Registry. Inclusion criteria consisted of fulfilling 2002/2016 SjS classification criteria, and exclusion criteria included chronic HCV/HIV infections and associated systemic autoimmune diseases. A statistical approach based on a directed acyclic graph was used, with all-cause and Sjögren-related mortality as primary endpoints. The key determinants that defined the disease phenotype at diagnosis (glandular, systemic, and immunological) were analysed as independent variables. FINDINGS: Between January 1st, 2014 and December 31, 2023, data from 11,372 patients with primary SjS (93.5% women, 78.4% classified as White, mean age at diagnosis of 51.1 years) included in the Registry were analysed. 876 (7.7%) deaths were recorded after a mean follow-up of 8.6 years (SD 7.12). Univariate analysis of prognostic factors for all-cause death identified eight Sjögren-related variables (ocular and oral tests, salivary biopsy, ESSDAI, ANA, anti-Ro, anti-La, and cryoglobulins). The multivariate CPH model adjusted for these variables and the epidemiological features showed that DAS-ESSDAI (high vs no high: HR = 1.68; 95% CI, 1.27–2.22) and cryoglobulins (positive vs negative: HR = 1.72; 95% CI, 1.22–2.42) were independent predictors of all-cause death. Of the 640 deaths with available information detailing the specific cause of death, 14% were due to systemic SjS. Univariate analysis of prognostic factors for Sjögren-cause death identified five Sjögren-related variables (oral tests, clinESSDAI, DAS-ESSDAI, ANA, and cryoglobulins). The multivariate competing risks CPH model adjusted for these variables and the epidemiological features showed that oral tests (abnormal vs normal results: HR = 1.38; 95% CI, 1.01–1.87), DAS-ESSDAI (high vs no high: HR = 1.55; 95% CI, 1.22–1.96) and cryoglobulins (positive vs negative: HR = 1.52; 95% CI, 1.16–2) were independent predictors of SjS-related death. INTERPRETATION: The key mortality risk factors at the time of SjS diagnosis were positive cryoglobulins and a high systemic activity scored using the ESSDAI, conferring a 2-times increased risk of all-cause and SjS-related death. ESSDAI measurement and cryoglobulin testing should be considered mandatory when an individual is diagnosed with SjS. FUNDING: 10.13039/100004336Novartis.
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spelling pubmed-103448112023-07-15 Mortality risk factors in primary Sjögren syndrome: a real-world, retrospective, cohort study Brito-Zerón, Pilar Flores-Chávez, Alejandra Horváth, Ildiko Fanny Rasmussen, Astrid Li, Xiaomei Olsson, Peter Vissink, Arjan Priori, Roberta Armagan, Berkan Hernandez-Molina, Gabriela Praprotnik, Sonja Quartuccio, Luca Inanç, Nevsun Özkızıltaş, Burcugül Bartoloni, Elena Sebastian, Agata Romão, Vasco C. Solans, Roser Pasoto, Sandra G. Rischmueller, Maureen Galisteo, Carlos Suzuki, Yasunori Trevisani, Virginia Fernandes Moça Fugmann, Cecilia González-García, Andrés Carubbi, Francesco Jurcut, Ciprian Shimizu, Toshimasa Retamozo, Soledad Atzeni, Fabiola Hofauer, Benedikt Melchor-Díaz, Sheila Gheita, Tamer López-Dupla, Miguel Fonseca-Aizpuru, Eva Giacomelli, Roberto Vázquez, Marcos Consani, Sandra Akasbi, Miriam Nakamura, Hideki Szántó, Antónia Farris, A. Darise Wang, Li Mandl, Thomas Gattamelata, Angelica Kilic, Levent Pirkmajer, Katja Perdan Abacar, Kerem Tufan, Abdurrahman de Vita, Salvatore Bootsma, Hendrika Ramos-Casals, Manuel eClinicalMedicine Articles BACKGROUND: What baseline predictors would be involved in mortality in people with primary Sjögren syndrome (SjS) remains uncertain. This study aimed to investigate the baseline characteristics collected at the time of diagnosis of SjS associated with mortality and to identify mortality risk factors for all-cause death and deaths related to systemic SjS activity measured by the ESSDAI score. METHODS: In this international, real-world, retrospective, cohort study, we retrospectively collected data from 27 countries on mortality and causes of death from the Big Data Sjögren Registry. Inclusion criteria consisted of fulfilling 2002/2016 SjS classification criteria, and exclusion criteria included chronic HCV/HIV infections and associated systemic autoimmune diseases. A statistical approach based on a directed acyclic graph was used, with all-cause and Sjögren-related mortality as primary endpoints. The key determinants that defined the disease phenotype at diagnosis (glandular, systemic, and immunological) were analysed as independent variables. FINDINGS: Between January 1st, 2014 and December 31, 2023, data from 11,372 patients with primary SjS (93.5% women, 78.4% classified as White, mean age at diagnosis of 51.1 years) included in the Registry were analysed. 876 (7.7%) deaths were recorded after a mean follow-up of 8.6 years (SD 7.12). Univariate analysis of prognostic factors for all-cause death identified eight Sjögren-related variables (ocular and oral tests, salivary biopsy, ESSDAI, ANA, anti-Ro, anti-La, and cryoglobulins). The multivariate CPH model adjusted for these variables and the epidemiological features showed that DAS-ESSDAI (high vs no high: HR = 1.68; 95% CI, 1.27–2.22) and cryoglobulins (positive vs negative: HR = 1.72; 95% CI, 1.22–2.42) were independent predictors of all-cause death. Of the 640 deaths with available information detailing the specific cause of death, 14% were due to systemic SjS. Univariate analysis of prognostic factors for Sjögren-cause death identified five Sjögren-related variables (oral tests, clinESSDAI, DAS-ESSDAI, ANA, and cryoglobulins). The multivariate competing risks CPH model adjusted for these variables and the epidemiological features showed that oral tests (abnormal vs normal results: HR = 1.38; 95% CI, 1.01–1.87), DAS-ESSDAI (high vs no high: HR = 1.55; 95% CI, 1.22–1.96) and cryoglobulins (positive vs negative: HR = 1.52; 95% CI, 1.16–2) were independent predictors of SjS-related death. INTERPRETATION: The key mortality risk factors at the time of SjS diagnosis were positive cryoglobulins and a high systemic activity scored using the ESSDAI, conferring a 2-times increased risk of all-cause and SjS-related death. ESSDAI measurement and cryoglobulin testing should be considered mandatory when an individual is diagnosed with SjS. FUNDING: 10.13039/100004336Novartis. Elsevier 2023-07-04 /pmc/articles/PMC10344811/ /pubmed/37457113 http://dx.doi.org/10.1016/j.eclinm.2023.102062 Text en © 2023 The Author(s) https://creativecommons.org/licenses/by/4.0/This is an open access article under the CC BY license (http://creativecommons.org/licenses/by/4.0/).
spellingShingle Articles
Brito-Zerón, Pilar
Flores-Chávez, Alejandra
Horváth, Ildiko Fanny
Rasmussen, Astrid
Li, Xiaomei
Olsson, Peter
Vissink, Arjan
Priori, Roberta
Armagan, Berkan
Hernandez-Molina, Gabriela
Praprotnik, Sonja
Quartuccio, Luca
Inanç, Nevsun
Özkızıltaş, Burcugül
Bartoloni, Elena
Sebastian, Agata
Romão, Vasco C.
Solans, Roser
Pasoto, Sandra G.
Rischmueller, Maureen
Galisteo, Carlos
Suzuki, Yasunori
Trevisani, Virginia Fernandes Moça
Fugmann, Cecilia
González-García, Andrés
Carubbi, Francesco
Jurcut, Ciprian
Shimizu, Toshimasa
Retamozo, Soledad
Atzeni, Fabiola
Hofauer, Benedikt
Melchor-Díaz, Sheila
Gheita, Tamer
López-Dupla, Miguel
Fonseca-Aizpuru, Eva
Giacomelli, Roberto
Vázquez, Marcos
Consani, Sandra
Akasbi, Miriam
Nakamura, Hideki
Szántó, Antónia
Farris, A. Darise
Wang, Li
Mandl, Thomas
Gattamelata, Angelica
Kilic, Levent
Pirkmajer, Katja Perdan
Abacar, Kerem
Tufan, Abdurrahman
de Vita, Salvatore
Bootsma, Hendrika
Ramos-Casals, Manuel
Mortality risk factors in primary Sjögren syndrome: a real-world, retrospective, cohort study
title Mortality risk factors in primary Sjögren syndrome: a real-world, retrospective, cohort study
title_full Mortality risk factors in primary Sjögren syndrome: a real-world, retrospective, cohort study
title_fullStr Mortality risk factors in primary Sjögren syndrome: a real-world, retrospective, cohort study
title_full_unstemmed Mortality risk factors in primary Sjögren syndrome: a real-world, retrospective, cohort study
title_short Mortality risk factors in primary Sjögren syndrome: a real-world, retrospective, cohort study
title_sort mortality risk factors in primary sjögren syndrome: a real-world, retrospective, cohort study
topic Articles
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC10344811/
https://www.ncbi.nlm.nih.gov/pubmed/37457113
http://dx.doi.org/10.1016/j.eclinm.2023.102062
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