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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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001-es BibID:	BIBFORM100740
035-os BibID:	(WOS)000671076800063 (Scopus)85108742062
Első szerző:	Brito-Zerón, Pilar
Cím:	SARS-CoV-2 infection in patients with primary Sjögren syndrome : characterization and outcomes of 51 patients / Brito-Zerón Pilar, Melchor Sheila, Seror Raphaele, Priori Roberta, Solans Roser, Kostov Belchin, Baldini Chiara, Carubbi Francesco, Callejas Jose Luis, Guisado-Vasco Pablo, Hernández-Molina Gabriela, Pasoto Sandra G., Valim Valeria, Sisó-Almirall Antoni, Mariette Xavier, Carreira Patricia, Ramos-Casals Manuel, Sjögren Big Data Consortium, EULAR-SS Task Force Big Data Consortium
Dátum:	2021
ISSN:	1462-0324 1462-0332
Megjegyzések:	Objective: To analyse the prognosis and outcomes of SARS-CoV-2 infection in patients with primary SS. Methods: We searched for patients with primary SS presenting with SARS-CoV-2 infection (defined following and according to the European Centre for Disease Prevention and Control guidelines) among those included in the Big Data Sjögren Registry, an international, multicentre registry of patients diagnosed according to the 2002/2016 classification criteria. Results: A total of 51 patients were included in the study (46 women, mean age at diagnosis of infection of 60 years). According to the number of patients with primary SS evaluated in the Registry (n = 8211), the estimated frequency of SARS-CoV-2 infection was 0.62% (95% CI 0.44, 0.80). All but two presented with symptoms suggestive of COVID-19, including fever (82%), cough (57%), dyspnoea (39%), fatigue/myalgias (27%) and diarrhoea (24%), and the most frequent abnormalities included raised lactate dehydrogenase (LDH) (88%), CRP (81%) and D-dimer (82%) values, and lymphopenia (70%). Infection was managed at home in 26 (51%) cases and 25 (49%) required hospitalization (five required admission to ICU, four died). Compared with patients managed at home, those requiring hospitalization had higher odds of having lymphopenia as laboratory abnormality (adjusted OR 21.22, 95% CI 2.39, 524.09). Patients with comorbidities had an older age (adjusted OR 1.05, 95% CI 1.00, 1.11) and showed a risk for hospital admission six times higher than those without (adjusted OR 6.01, 95% CI 1.72, 23.51) in the multivariate analysis. Conclusion: Baseline comorbidities were a key risk factor for a more complicated COVID-19 in patients with primary SS, with higher rates of hospitalization and poor outcomes in comparison with patients without comorbidities.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk Primary SS COVID-19 SARS-Cov-2 comorbidities outcomes
Megjelenés:	Rheumatology. - 60 : 6 (2021), p. 2946-2957. -
További szerzők:	Melchor, Sheila Seror, Raphaele Priori, Roberta Solans, Roser Kostov, Belchin Baldini, Chiara Carubbi, Francesco Callejas, J. L. Guisado-Vasco, Pablo Hernandez-Molina, Gabriela Pasoto, Sandra Valim, Valeria Sisó-Almirall, Antoni Mariette, Xavier Carreira, Patricia E. Ramos-Casals, Manuel Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Tarr Tünde (1976-) (belgyógyász, allergológus és klinikai immunológus) Sjögren Big Data Consortium EULAR-SS Task Force Big Data Consortium
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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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001-es BibID:	BIBFORM100734
035-os BibID:	(WOS)000731864300024 (Scopus)85122843096
Első szerző:	Retamozo, Soledad
Cím:	Influence of the age at diagnosis in the disease expression of primary Sjögren syndrome. Analysis of 12,753 patients from the Sjögren Big Data Consortium / S. Retamozo, N. Acar-Denizli, I. F. Horváth, W. F. Ng, A. Rasmussen, X. Dong, X. Li, C. Baldini, P. Olsson, R. Priori, R. Seror, Jacques-Eric Gottenberg, A. A. Kruize, G. Hernandez-Molina, A. Vissink, P. Sandhya, B. Armagan, L. Quartuccio, A. Sebastian, S. Praprotnik, E. Bartoloni, Seung-Ki Kwok, M. Kvarnstrom, M. Rischmueller, R. Soláns-Laqué, D. Sene, S. G. Pasoto, Y. Suzuki, D. A. Isenberg, V. Valim, G. Nordmark, H. Nakamura, V. Fernandes Moca Trevisani, B. Hofauer, A. Sisó-Almirall, R. Giacomelli, V. Devauchelle-Pensec, M. Bombardieri, F. Atzeni, D. Hammenfors, B. Maure, S. E. Carsons, T. Gheita, I. Sánchez-Berná, M. López-Dupla, J. Morel, N. Inanc, E. Fonseca-Aizpuru, C. Morcillo, C. Vollenweider, S. Melchor, M. Vázquez, E. Díaz-Cuiza, S. Consani-Fernández, B. De-Miguel-Campo, A. Szántó, S. Bombardieri, A. Gattamelata, A. Hinrichs, J. Sánchez-Guerrero, D. Danda, L. Kilic, S. De Vita, P. Wiland, R. Gerli, S. H. Park, M. Wahren-Herlenius, H. Bootsma, X. Mariette, M. Ramos-Casals, P. Brito-Zerón
Dátum:	2021
ISSN:	0392-856X
Megjegyzések:	Objectives: To analyse how the main components of the disease phenotype (sicca symptoms, diagnostic tests, immunological markers and systemic disease) can be driven by the age at diagnosis of primary Sjögren's syndrome (pSS). Methods: By January 2021, the participant centres had included 12,753 patients from 25 countries that fulfilled the 2002/2016 classification criteria for pSS. The age at diagnosis was defined as the time when the attending physician confirmed fulfilment of the criteria. Patients were clustered according to age at diagnosis. 50 clusters with more than 100 observations (from 27 to 76 years) were used to study the influence of the age at diagnosis in the disease expression. Results: There was a consistent increase in the frequency of oral dryness according to the age at diagnosis, with a frequency of <90% in patients diagnosed at the youngest ages and >95% in those diagnosed at the oldest ages. The smooth curves that best fitted a linear model were the frequency of dry mouth (adjusted R2 0.87) and the frequency of abnormal oral tests (adjusted R2 0.72). Therefore, for each 1-year increase in the age at diagnosis, the frequency of dry mouth increased by 0.13%, and the frequency of abnormal oral diagnostic tests by 0.11%. There was a consistent year-by-year decrease in the frequency of all autoantibodies and immunological markers except for cryoglobulins. According to the linear models, for each 1-year increase in the age at diagnosis, the frequency of a positive result decreased by 0.57% (for anti-Ro antibodies), 0.47% (for RF) and 0.42% (for anti-La antibodies). The ESSDAI domains which showed a more consistent decrease were glandular and lymph node involvement (for each 1-year increase in the age at diagnosis, the frequency of activity decreased by 0.18%), and constitutional, cutaneous, and haematological involvements (the frequency decreased by 0.09% for each 1-year increase). In contrast, other domains showed an ascending pattern, especially pulmonary involvement (for each 1-year increase in the age at diagnosis, the frequency of activity increased by 0.22%), and peripheral nerve involvement (the frequency increased by 0.09% for each 1-year increase). Conclusions: The influence of the age at diagnosis on the key phenotypic features of pSS is strong, and should be considered critical not only for designing a personalised diagnostic approach, but also to be carefully considered when analysing the results of diagnostic tests and immunological parameters, and when internal organ involvement is suspected at diagnosis.
Tárgyszavak:	Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban folyóiratcikk Sjögren's syndrome age disease phenotype immunological markers
Megjelenés:	Clinical and Experimental Rheumatology. - 39 : 6 (2021), p. 166-174. -
További szerzők:	Acar-Denizli, Nihan Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Ng, Wan Fai Rasmussen, Astrid Dong, X. Li, X. Baldini, Chiara Olsson, Peter Priori, Roberta Seror, Raphaele Gottenberg, Jacques-Eric Kruize, Aike A. Hernandez-Molina, Gabriela Vissink, Arjan Sandhya, Pulukool Armagan, Berkan Quartuccio, Luca Sebastian, Agata Praprotnik, Sonja Bartoloni, Elena Kwok, Seung-Ki Kvarnstrom, Marika Rischmueller, Maureen Soláns-Laqué, Roser Sene, Damien Pasoto, Sandra Suzuki, Yasunori Isenberg, David A. Valim, Valeria Nordmark, Gunnel Nakamura, Hideki Fernandes Moça Trevisani, Virginia Hofauer, Benedikt Sisó-Almirall, Antoni Giacomelli, Roberto Devauchelle-Pensec, Valerie Bombardieri, Michele Atzeni, F. Hammenfors, Daniel Maure, B. Carsons, Steven E. Gheita, Tamer A. Sánchez-Berná, I. López-Dupla, Miguel Morel, Jacques Inanc, Nevsun Fonseca-Aizpuru, Eva Morcillo, C. Vollenveider, Cristina Melchor, Sheila Vázquez, Marta Diaz-Cuiza, E. Consani-Fernández, S. de-Miguel-Campo, B. Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Bombardieri, Stefano Gattamelata, Angelica Hinrichs, Anneline Sanchez-Guerrero, Jorge Danda, Debashish Kilic, Levent Vita, Salvatore de Wiland, Piotr Gerli, Roberto Park, S. H. Wahren-Herlenius, Marie Bootsma, Hendrika Mariette, Xavier Ramos-Casals, Manuel Brito-Zerón, Pilar
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