Mortality risk factors in primary Sjögren syndrome : a real-world, retrospective, cohort study
(2023) In EClinicalMedicine 61. p.102062-102062- Abstract
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... (More)
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: Novartis.
(Less)
- author
- author collaboration
- organization
- publishing date
- 2023-07
- type
- Contribution to journal
- publication status
- published
- subject
- in
- EClinicalMedicine
- volume
- 61
- article number
- 102062
- pages
- 102062 - 102062
- publisher
- Lancet Publishing Group
- external identifiers
-
- scopus:85164311201
- pmid:37457113
- ISSN
- 2589-5370
- DOI
- 10.1016/j.eclinm.2023.102062
- language
- English
- LU publication?
- yes
- additional info
- © 2023 The Author(s).
- id
- 74639d98-6f72-43bd-91de-4c25e8119d8d
- date added to LUP
- 2023-09-13 15:35:09
- date last changed
- 2024-04-20 03:13:54
@article{74639d98-6f72-43bd-91de-4c25e8119d8d,
abstract = {{<p>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.</p><p>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.</p><p>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.</p><p>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.</p><p>FUNDING: Novartis.</p>}},
author = {{Brito-Zerón, Pilar and Flores-Chávez, Alejandra and Horváth, Ildiko Fanny and Rasmussen, Astrid and Li, Xiaomei and Olsson, Peter and Vissink, Arjan and Priori, Roberta and Armagan, Berkan and Hernandez-Molina, Gabriela and Praprotnik, Sonja and Quartuccio, Luca and Inanç, Nevsun and Özkızıltaş, Burcugül and Bartoloni, Elena and Sebastian, Agata and Romão, Vasco C and Solans, Roser and Pasoto, Sandra G and Rischmueller, Maureen and Galisteo, Carlos and Suzuki, Yasunori and Trevisani, Virginia Fernandes Moça and Fugmann, Cecilia and González-García, Andrés and Carubbi, Francesco and Jurcut, Ciprian and Shimizu, Toshimasa and Retamozo, Soledad and Atzeni, Fabiola and Hofauer, Benedikt and Melchor-Díaz, Sheila and Gheita, Tamer and López-Dupla, Miguel and Fonseca-Aizpuru, Eva and Giacomelli, Roberto and Vázquez, Marcos and Consani, Sandra and Akasbi, Miriam and Nakamura, Hideki and Szántó, Antónia and Farris, A Darise and Wang, Li and Mandl, Thomas and Gattamelata, Angelica and Kilic, Levent and Pirkmajer, Katja Perdan and Abacar, Kerem and Tufan, Abdurrahman and de Vita, Salvatore and Bootsma, Hendrika}},
issn = {{2589-5370}},
language = {{eng}},
pages = {{102062--102062}},
publisher = {{Lancet Publishing Group}},
series = {{EClinicalMedicine}},
title = {{Mortality risk factors in primary Sjögren syndrome : a real-world, retrospective, cohort study}},
url = {{http://dx.doi.org/10.1016/j.eclinm.2023.102062}},
doi = {{10.1016/j.eclinm.2023.102062}},
volume = {{61}},
year = {{2023}},
}