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001-es BibID:BIBFORM135721
035-os BibID:(scopus)105025172537
Első szerző:Brito-Zerón, Pilar
Cím:Sex disparities in the phenotype at diagnosis of Sjögren's disease : artificial intelligence-driven characterisation in 17,416 patients / Brito-Zerón Pilar, Flores-Chávez Alejandra, Horváth Ildiko Fanny, Priori Roberta, Bootsma Hendrika, Armagan Berkan, Quartuccio Luca, Praprotnik Sonja, Suzuki Yasonuri, Hernandez-Molina Gabriela, Romao Vasco C., Sebastian Agata, Bartoloni Elena, Rischmueller Maureen, Solans Roser, Pasoto Sandra G., Nordmark Gunnel, Sánchez Berná Isabel, Carubbi Francesco, Fernandes Moca Trevisani Virginia, Valim Valeria, Melchor Sheila, Maure Noia Brenda, Fonseca-Aizpuru Eva, Delgado Lucía, Nakamura Hideki, López-Dupla Miguel, Vazquez Marcos, Akasbi Miriam, Policarpo Torres Guillem, De Miguel Campo Borja, Rouco Rosana, Szántó Antónia, Gattamelata Angelica, Vissink Arjan, Kilic Levent, Manfre Valeria, Perdan Pirkmajer Katja, Fujisawa Yuhei, Pereira da Costa Roberto, Wiland Piotr, Gerli Roberto, Kirana Chandra, Nardi Norma, Ramos-Casals Manuel, Sjögren Big Data Consortium
Dátum:2025
ISSN:0392-856X 1593-098X
Megjegyzések:Objectives: Sjögren disease (SjD) predominantly affects females, but the early disease presentation in male patients remains poorly characterised due to historically small sample sizes. The aim of this study was to investigate sex?based differences in the clinical phenotype at diagnosis of SjD and identify predictors of patient sex using a large international cohort and AI?enhanced analysis. Methods: Cross-sectional analysis of an anonymised dataset comprising 17,416 worldwide patients fulfilling the 2002/2016 classification criteria (Sjögren Big Data Registry). We stratified the dataset by sex and conducted a comparative analysis of baseline glandular and systemic involvement, organ-specific ESSDAI domains, and immunological profiles. Multivariate logistic regression models were developed, adjusting for epidemiological confounders (age and ethnicity) to identify predictors of sex classification. We used a generative AI (OpenAI's GPT-4o model) environment with Python (version 3.9) and the pandas (1.4.3), numpy (1.21.5), and matplotlib (3.5.1) libraries. All analyses adhered to GDPR standards, with anonymized patient data and strictly controlled secure environments. Results: The cohort included 1,161 (6.67%) men and 16,255 (93.33%) women, with a mean age at diagnosis of 51.11 years (SD=14.45). Men showed a higher mean age at diagnosis (54.09 vs. 51.42 years in women; t=6.08, p<0.0001), a higher average ESSDAI score (7.65 vs. 5.93; t=7.91, p<0.0001) and higher frequencies in severe DAS categories (i.e. high activity 20% vs. 12% in women, ?? = 81.15, p<0.0001). The epidemiologically-adjusted logistic regression model (pseudo R-squared value of 0.026) identified statistical significance for age (coefficient =0.009, p=0.024; each additional year in age increased the likelihood of being female by 1.4%), ethnicity (coefficient=0.579, HR=1.78, p=0.004), ocular dryness (coefficient=-0.607, HR=0.54, p<0.001), and systemic activity in the glandular (coefficient=0.359, HR=1.43, p=0.006) and pulmonary (coefficient=0.445, HR=1.56, p=0.004) ESSDAI domains. Conclusions: Male SjD patients present a distinct, more systemic phenotype at diagnosis. Awareness of sex?specific features can improve early recognition and tailored management.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's disease
Sex disparities
phenotype at diagnosis
artificial intelligence
big data
Megjelenés:Clinical And Experimental Rheumatology. - 43 : 12 (2025), p. 2133-2141. -
További szerzők:Flores-Chávez, Alejandra Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Priori, Roberta Bootsma, Hendrika Armagan, Berkan Quartuccio, Luca Praprotnik, Sonja Suzuki, Yasunori Hernandez-Molina, Gabriela Romão, Vasco C. Sebastian, Agata Bartoloni, Elena Rischmueller, Maureen Solans, Roser Pasoto, Sandra Nordmark, Gunnel Sánchez Berná, Isabel Carubbi, Francesco Fernandes Moça Trevisani, Virginia Valim, Valeria Melchor, Sheila Maure, B. Fonseca-Aizpuru, Eva Delgado, Lucía Nakamura, Hideki López-Dupla, Miguel Vázquez, Marcos Akasbi, Miriam Policarpo Torres, Guillem De Miguel Campo, Borja Rouco, Rosana Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Gattamelata, Angelica Vissink, Arjan Kilic, Levent Manfre, Valeria Perdan Pirkmajer, Katja Fujisawa, Yuhei Pereira da Costa, Roberto Wiland, Piotr Gerli, Roberto Kirana, Chandra Nardi, Norma Ramos-Casals, Manuel Sjögren Big Data Consortium
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2.

001-es BibID:BIBFORM115775
035-os BibID:(scopus)85164311201 (wos)001040655800001
Első szerző:Brito-Zerón, Pilar
Cím: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, Inanc Nevsun, Özkiziltas Burcugül, Bartoloni Elena, Sebastian Agata, Romao Vasco C., Solans Roser, Pasoto Sandra G., Rischmueller Maureen, Galisteo Carlos, Suzuki Yasunori, Trevisani Virginia Fernandes Moca, 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, Sjögren Big Data Consortium
Dátum:2023
ISSN:2589-5370
Megjegyzések: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.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Cardiovascular
Infection
Lymphoma
Mortality
Sjögren syndrome
Systemic disease
Megjelenés:eClinicalMedicine. - 61 (2023), p. 1-16. -
További szerzők:Flores-Chávez, Alejandra Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Rasmussen, Astrid Li, Xiaomei Olsson, Peter Vissink, Arjan Priori, Roberta Armagan, Berkan Hernandez-Molina, Gabriela Praprotnik, Sonja Quartuccio, Luca Inanc, Nevsun Özkiziltaș, Burcugül Bartoloni, Elena Sebastian, Agata Romão, Vasco C. Solans, Roser Pasoto, Sandra 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, S. Gheita, Tamer A. López-Dupla, Miguel Fonseca-Aizpuru, Eva Giacomelli, Roberto Vázquez, Marcos Consani, Sandra Akasbi, Miriam Nakamura, Hideki Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Farris, Darise Wang, Li Mandl, Thomas Gattamelata, Angelica Kilic, Levent Pirkmajer, Katja Perdan Abacar, Kerem Tufan, Abdurrahman Vita, Salvatore de Bootsma, Hendrika Ramos-Casals, Manuel Sjögren Big Data Consortium
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3.

001-es BibID:BIBFORM100733
035-os BibID:(WOS)000731864300009 (Scopus)85122843135
Első szerző:Brito-Zerón, Pilar
Cím:Post-COVID-19 syndrome in patients with primary Sjögren's syndrome after acute SARS-CoV-2 infection / P. Brito-Zerón, N. Acar-Denizli, V. C. Romão, B. Armagan, R. Seror, F. Carubbi, S. Melchor, R. Priori, V. Valim, S. Retamozo, S. G. Pasoto, V. F. M. Trevisani, B. Hofauer, A. Szántó, N. Inanc, G. Hernández-Molina, A. Sebastian, E. Bartoloni, V. Devauchelle-Pensec, M. Akasbi, F. Giardina, M. Bandeira, A. Sisó-Almirall, M. Ramos-Casals, Sjögren Big Data Consortium
Dátum:2021
ISSN:0392-856X
Megjegyzések:OBJECTIVES: To analyse the frequency and characteristics of post-COVID-19 syndrome in patients with primary Sjögren's syndrome (pSS) affected by acute SARS-CoV-2 infection. METHODS: By the first week of April 2021, all centres included in the Big Data Sjögren Consortium were contacted asking for patients included in the Registry diagnosed with SARSCoV-2 infection according to the ECDC guidelines. According to the NICE definitions, symptoms related to COVID-19 were classified as acute COVID-19 (signs and symptoms for up to 4 weeks), ongoing symptomatic COVID-19 (presence of signs and symptoms from 4 to 12 weeks) and post-COVID-19 syndrome (signs and symptoms that continue for > 12 weeks not explained by an alternative diagnosis after a protocolized study). RESULTS: We identified 132 patients who were followed a mean follow-up of 137.8 days (ranging from 5 days to 388 days) after being diagnosed with COVID-19. In the last visit, 75 (57%) patients remained symptomatic: 68 (52%) remained symptomatic for more than 4 weeks fulfilling the NICE definition for ongoing symptomatic post-COVID-19, and 38 (29%) remained symptomatic for more than 12 weeks fulfilling the definition of post-COVID-19 syndrome. More than 40% of pSS patients reported the persistence of four symptoms or more, including anxiety/depression (59%), arthralgias (56%), sleep disorder (44%), fatigue (40%), anosmia (34%) and myalgias (32%). Age-sex adjusted multivariate analysis identified raised LDH levels (OR 10.36), raised CRP levels (OR 7.33), use of hydroxychloroquine (OR 3.51) and antiviral agents (OR 3.38), hospital admission (OR 8.29), mean length of hospital admission (OR 1.1) and requirement of supplemental oxygen (OR 6.94) as factors associated with a higher risk of developing post-COVID-19 syndrome. A sensitivity analysis including hospital admission in the adjusted model confirmed raised CRP levels (OR 8.6, 95% CI 1.33-104.44) and use of hydroxychloroquine (OR 2.52, 95% CI 1.00-6.47) as the key independent factors associated with an enhanced risk of developing post-COVID-19 syndrome. CONCLUSIONS: This is the first study that analyses the frequency and characteristics of post-COVID-19 syndrome in patients affected by a systemic autoimmune disease. We found that 57% of patients with pSS affected by COVID-19 remain symptomatic after a mean follow-up of 5 months. The risk of developing post-COVID-19 syndrome in patients who required hospitalisation was 8-times higher than in non-hospitalised patients, with baseline raised CRP levels and the use of hydroxychloroquine being independent risk factors for post-COVID-19.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
post-COVID-19 syndrome
primary Sjögren's syndrome
SARS-CoV-2
long COVID-19
hydroxychloroquine
hospital admission
Megjelenés:Clinical and Experimental Rheumatology. - 39 : 6 (2021), p. 57-65. -
További szerzők:Acar-Denizli, Nihan Romão, Vasco C. Armagan, Berkan Seror, Raphaele Carubbi, Francesco Melchor, Sheila Priori, Roberta Valim, Valeria Retamozo, Soledad Pasoto, Sandra Trevisani, Virginia Fernandes Moça Hofauer, Benedikt Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Inanc, Nevsun Hernandez-Molina, Gabriela Sebastian, Agata Bartoloni, Elena Devauchelle-Pensec, Valerie Akasbi, Miriam Giardina, Federico Bandeira, Matilde Sisó-Almirall, Antoni Ramos-Casals, Manuel Sjögren Big Data Consortium
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4.

001-es BibID:BIBFORM135724
035-os BibID:(scopus)105025172219
Első szerző:Delgado, Lucía
Cím:Immunological signatures in patients with Sjögren's disease : association with systemic disease activity at diagnosis / Delgado Lucía, Flores-Chávez Alejandra, Szántó Antónia, Priori Roberta, Bootsma Hendrika, Armagan Berkan, Quartuccio Luca, Praprotnik Sonja, Suzuki Yasonuri, Hernandez-Molina Gabriela, Romao Vasco C., Sebastian Agata, Bartoloni Elena, Rischmueller Maureen, Solans Roser, Pasoto Sandra G., Fugmann Cecilia, Sánchez Berná Isabel, Carubbi Francesco, Fernandes Moca Trevisani Virginia, Valim Valeria, Melchor Sheila, Maure Noia Brenda, Fonseca-Aizpuru Eva, Nakamura Hideki, López-Dupla Miguel, Vazquez Marcos, Akasbi Miriam, Policarpo Torres Guillem, De Miguel Campo Borja, Suru Mihaela Roxana, Vericat Queralt Carmen, Horváth Ildiko Fanny, Fischetti Ilenia, Vissink Arjan, Kilic Levent, Manfre Valeria, Perdan Pirkmajer Katja, Fujisawa Yuhei, Bandeira Matilde, Proc Krzysztof, Gerli Roberto, Kirana Chandra, Nardi Norma, Ramos-Casals Manuel, Brito-Zerón Pilar, Sjögren Big Data Consortium
Dátum:2025
ISSN:0392-856X 1593-098X
Megjegyzések:OBJECTIVES: This study aimed to analyse the relationship between distinct autoantibody combinations (immunological signatures) and systemic disease activity in patients with Sjögren's disease (SjD). The hypothesis was that specific multi-autoantibody signatures would be associated with higher systemic disease activity at diagnosis, serving as predictors of a more severe disease course. METHODS: A retrospective observational study was conducted using data from the Big Data Sjögren Project Consortium, an international multicentre registry. The serological status (positive/negative) at diagnosis for ANA, RF, anti-Ro, and anti-La was recorded for each patient. Systemic disease activity was assessed using the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) and a simplified Disease Activity Score (DAS) categorised as low, moderate, or high. Statistical analyses included pairwise comparisons, a sensitivity analysis grouping signatures by the number of positive antibodies, and demographic-adjusted ordinal models. RESULTS: Serum autoantibodies were highly prevalent, with over 94% of patients having at least one autoantibody. The mean ESSDAI values varied significantly across signatures. The fully seronegative group had the lowest mean ESSDAI at 3.61, while the fully seropositive group (ANA+/Ro+/La+/RF+) had the highest among common phenotypes, with a mean of 7.93. A strong dose-response relationship was observed, with each additional positive autoantibody associated with a 1.11-point mean increase in ESSDAI and a 35% increase in the odds of being in a higher DAS category. The rarest signatures, such as ANA?/Ro?/La+/RF+, exhibited the highest mean systemic activity (mean 13.20). CONCLUSIONS: The number and combination of SjD-related autoantibodies at diagnosis are robustly associated with systemic disease activity. Multi-positive profiles, particularly those combining RF with anti-Ro, identify patients at higher risk of systemic activity. Interpreting combined serological patterns offers an immediate, low-cost method for patient stratification and can help guide clinical management.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's disease
Sjögren's syndrome
ESSDAI
EULAR
autoantibodies
Immunological signatures
rheumatoid factor
anti-Ro
anti-La
ANA
Systemic disease activity
Megjelenés:Clinical And Experimental Rheumatology. - 43 : 12 (2025), p. 2124-2132. -
További szerzők:Flores-Chávez, Alejandra Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Priori, Roberta Bootsma, Hendrika Armagan, Berkan Quartuccio, Luca Praprotnik, Sonja Suzuki, Yasunori Hernandez-Molina, Gabriela Romão, Vasco C. Sebastian, Agata Bartoloni, Elena Rischmueller, Maureen Solans, Roser Pasoto, Sandra Fugmann, Cecilia Sánchez Berná, Isabel Carubbi, Francesco Fernandes Moça Trevisani, Virginia Valim, Valeria Melchor, Sheila Maure, B. Fonseca-Aizpuru, Eva Nakamura, Hideki López-Dupla, Miguel Vázquez, Marcos Akasbi, Miriam Policarpo Torres, Guillem De Miguel Campo, Borja Suru, Mihaela Roxana Vericat Queralt, Carmen Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Fischetti, Ilenia Vissink, Arjan Kilic, Levent Manfre, Valeria Perdan Pirkmajer, Katja Fujisawa, Yuhei Bandeira, Matilde Proc, Krzysztof Gerli, Roberto Kirana, Chandra Nardi, Norma Ramos-Casals, Manuel Brito-Zerón, Pilar Sjögren Big Data Consortium
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