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

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

Brito-Zerón, Pilar (författare): Sanitas Digital Hosp, Autoimmune Dis Unit, Res & Innovat Grp Autoimmune Dis, Hosp CIMA Ctr Med Milenium Balmes Snitas, Barcelona, Spain.,Hospital CIMA

Flores-Chávez, Alejandra (författare): Hosp Clin Barcelona, Dept Autoimmune Dis, ICMiD, Barcelona, Spain.,Hospital Clínic of Barcelona

Horváth, Ildiko Fanny (författare): Univ Debrecen, Fac Med, Div Clin Immunol, Debrecen, Hungary.,University of Debrecen

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Rasmussen, Astrid (författare): Oklahoma Med Res Fdn, Genes & Human Dis Res Program, Oklahoma City, OK USA.,Oklahoma Medical Research Foundation

Li, Xiaomei (författare): Univ Sci & Technol China, Affiliated Hosp USTC 1, Dept Rheumatol & Immunol, Anhui Prov Hosp, Hefei, Peoples R China.,University of Science and Technology of China

Olsson, Peter (författare): Lund University,Lunds universitet,Reumatologi,Forskargrupper vid Lunds universitet,Rheumatology,Lund University Research Groups,Skåne University Hospital

Vissink, Arjan (författare): Univ Groningen, Univ Med Ctr Groningen, Dept Oral & Maxillofacial Surger, Groningen, Netherlands.,University of Groningen

Priori, Roberta (författare): Sapienza Univ Rome, Dept Internal Med & Med Specialties, Rheumatol Clin, Rome, Italy.;St Camillus Int Univ Hlth Sci, UniCamillus, Rome, Italy.,Sapienza University of Rome

Armagan, Berkan (författare): Hacettepe Univ, Fac Med, Dept Internal Med, Div Rheumatol, Ankara, Turkiye.,Hacettepe University

Hernandez-Molina, Gabriela (författare): Inst Nacl Ciencias Med & Nutr Salvador Zubrican, Immunol & Rheumatol Dept, Mexico City, Mexico.,Instituto Nacional de Ciencias Médicas y Nutrición

Praprotnik, Sonja (författare): Univ Med Ctr, Dept Rheumatol, Ljubljana, Slovenia.,University Medical Centre Ljubljana

Quartuccio, Luca (författare): Univ Hosp St Maria Misericordia, Dept Med & Biol Sci, Clin Rheumatol, Udine, Italy.

Inanc, Nevsun (författare)

Özkiziltas, Burcugül (författare): Gazi Univ, Dept Internal Med, Div Rheumatol, Sch Med, Ankara, Turkiye.

Bartoloni, Elena (författare): Univ Perugia, Dept Med, Rheumatol Unit, Perugia, Italy.

Sebastian, Agata (författare): Wroclaw Med Hosp, Dept Rheumatol & Internal Med, Wroclaw, Poland.

Romão, Vasco C. (författare): Ctr Hosp Univ Lisboa Norte, Lisbon, Portugal.

Solans, Roser (författare): Hosp Valle De Hebron, Dept Internal Med, Barcelona, Spain.

Pasoto, Sandra G. (författare): Univ Sao Paulo, Hosp Clin HCFMUSP, Rheumatol Div, Fac Med, Sao Paulo, Brazil.

Rischmueller, Maureen (författare): Univ Adelaide, Queen Elizabeth Hosp, Dept Rheumatol, Discipline Med, Adelaide, SA, Australia.

Galisteo, Carlos (författare): Hosp Parc Tauli, Dept Rheumatol, Barcelona, Spain.

Suzuki, Yasunori (författare): Kanazawa Univ Hosp, Div Rheumatol, Kanazawa, Ishikawa, Japan.

Fernandes Moca Trevisani, Virginia (författare): Univ Fed Sao Paulo, Div Hlth Based Evidence, Sao Paulo, Brazil.

Fugmann, Cecilia (författare): Uppsala universitet,Reumatologi

González-García, Andrés (författare): Hosp 12 Octubre, Dept Rheumatol, Madrid, Spain.

Carubbi, Francesco (författare): San Salvatore Hosp, Dept Med, Internal Med & Nephrol Unit, Sulmona, Laquila, Italy.

Jurcut, Ciprian (författare): Carol Davila Cent Mil Emergency Hosp, Dept Internal Med, Bucharest, Romania.

Shimizu, Toshimasa (författare): Nagasaki Univ, Dept Immunol & Rheumatol, Div Adv Prevent Med Sci, Grad Sch Biomed Sci, Nagasaki, Japan.

Retamozo, Soledad (författare): Hosp Quiron Salud, Dept Rheumatol, Barcelona, Spain.

Atzeni, Fabiola (författare): Univ Messina, IRCCS Galeazzi Orthoped Inst, Milan & Rheumatol Unit, Messina, Italy.

Hofauer, Benedikt (författare): Tech Univ Munich, Otorhinolaryngol Head & Neck Surg, Munich, Germany.

Melchor-Díaz, Sheila (författare): Hosp 12 Octubre, Dept Rheumatol, Madrid, Spain.

Gheita, Tamer (författare): Cairo Univ, Kasr Al Ainy Sch Med, Rheumatol Dept, Cairo, Egypt.

López-Dupla, Miguel (författare): Hosp Joan 23, Dept Internal Med, Tarragona, Spain.

Fonseca-Aizpuru, Eva (författare): Hosp Cabuenes, Dept Internal Med, Gijon, Spain.

Giacomelli, Roberto (författare): Univ Aquila, Sch Med, Clin Unit Rheumatol, Laquila, Italy.

Vázquez, Marcos (författare): Hosp Clin, Dept Rheumatol, San Lorenzo, Paraguay.

Consani, Sandra (författare): Univ Republ UdelaR, Hosp Maciel, Internal Med, Montevideo, Uruguay.

Akasbi, Miriam (författare): Hosp Infanta Leonor, Dept Internal Med, Madrid, Spain.

Nakamura, Hideki (författare): Nihon Univ, Dept Med, Div Hematol & Rheumatol, Sch Med, Oyaguchi Kami Cho, Tokyo, Tokyo, Japan.

Szántó, Antónia (författare): Univ Debrecen, Fac Med, Div Clin Immunol, Debrecen, Hungary.

Farris, A. Darise (författare): Oklahoma Med Res Fdn, Genes & Human Dis Res Program, Oklahoma City, OK USA.

Wang, Li (författare): Univ Sci & Technol China, Affiliated Hosp USTC 1, Dept Rheumatol & Immunol, Anhui Prov Hosp, Hefei, Peoples R China.

Mandl, Thomas (författare): Lund University,Lunds universitet,Reumatologi,Forskargrupper vid Lunds universitet,Rheumatology,Lund University Research Groups,Skåne University Hospital

Gattamelata, Angelica (författare): Sapienza Univ Rome, Dept Internal Med & Med Specialties, Rheumatol Clin, Rome, Italy.

Kilic, Levent (författare): Hacettepe Univ, Fac Med, Dept Internal Med, Div Rheumatol, Ankara, Turkiye.

Perdan Pirkmajer, Katja (författare): Univ Med Ctr, Dept Rheumatol, Ljubljana, Slovenia.

Abacar, Kerem (författare): Marmara Univ, Sch Med, Istanbul, Turkiye.

Tufan, Abdurrahman (författare): Gazi Univ, Dept Internal Med, Div Rheumatol, Sch Med, Ankara, Turkiye.

de Vita, Salvatore (författare): Univ Hosp St Maria Misericordia, Dept Med & Biol Sci, Clin Rheumatol, Udine, Italy.

Bootsma, Hendrika (författare): Univ Groningen, Univ Med Ctr Groningen, Dept Rheumatol & Clin Immunol, Groningen, Netherlands.

Ramos-Casals, Manuel (författare): Hosp Clin Barcelona, Dept Autoimmune Dis, ICMiD, Barcelona, Spain.

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Sanitas Digital Hosp, Autoimmune Dis Unit, Res & Innovat Grp Autoimmune Dis, Hosp CIMA Ctr Med Milenium Balmes Snitas, Barcelona, Spain Hospital CIMA (creator_code:org_t)

Elsevier, 2023
2023
Engelska.
Ingår i: eClinicalMedicine. - : Elsevier. - 2589-5370. ; 61

Relaterad länk:: https://doi.org/10.1...; visa fler...; https://uu.diva-port... (primary) (Raw object); http://dx.doi.org/10... (free); https://urn.kb.se/re...; https://doi.org/10.1...; https://lup.lub.lu.s...; visa färre...

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BackgroundWhat 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.MethodsIn 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.FindingsBetween 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.InterpretationThe 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.

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Av författaren/redakt...: Brito-Zerón, Pil ...; Flores-Chávez, A ...; Horváth, Ildiko ...; Rasmussen, Astri ...; Li, Xiaomei; Olsson, Peter; visa fler...; Vissink, Arjan; Priori, Roberta; Armagan, Berkan; Hernandez-Molina ...; Praprotnik, Sonj ...; Quartuccio, Luca; Inanc, Nevsun; Özkiziltas, Burc ...; Bartoloni, Elena; Sebastian, Agata; Romão, Vasco C.; Solans, Roser; Pasoto, Sandra G ...; Rischmueller, Ma ...; Galisteo, Carlos; Suzuki, Yasunori; Fernandes Moca T ...; Fugmann, Cecilia; González-García, ...; Carubbi, Frances ...; Jurcut, Ciprian; Shimizu, Toshima ...; Retamozo, Soleda ...; Atzeni, Fabiola; Hofauer, Benedik ...; Melchor-Díaz, Sh ...; Gheita, Tamer; López-Dupla, Mig ...; Fonseca-Aizpuru, ...; Giacomelli, Robe ...; Vázquez, Marcos; Consani, Sandra; Akasbi, Miriam; Nakamura, Hideki; Szántó, Antónia; Farris, A. Daris ...; Wang, Li; Mandl, Thomas; Gattamelata, Ang ...; Kilic, Levent; Perdan Pirkmajer ...; Abacar, Kerem; Tufan, Abdurrahm ...; de Vita, Salvato ...; Bootsma, Hendrik ...; Ramos-Casals, Ma ...; visa färre...

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

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