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001-es BibID:BIBFORM091875
035-os BibID:(WOS)000581183900014 (Scopus)85094685088
Első szerző:Acar-Denizli, Nihan
Cím:Systemic phenotype related to primary Sjögren's syndrome in 279 patients carrying isolated anti-La/SSB antibodies / N. Acar-Denizli, I. F. Horváth, T. Mandl, R. Priori, A. Vissink, G. Hernandez-Molina, B. Armagan, S. Praprotnik, A. Sebastian, E. Bartoloni, M. Rischmueller, S. G. Pasoto, G. Nordmark, H. Nakamura, V. Fernandes Moça Trevisani, S. Retamozo, S. E. Carsons, B. Maure-Noia, I. Sánchez-Berná, M. López-Dupla, E. Fonseca-Aizpuru, S. Melchor Díaz, M. Vázquez, P. E. Díaz Cuiza, B. de Miguel Campo, W. F. Ng, A. Rasmussen, X. Dong, X. Li, C. Baldini, R. Seror, Jacques-Eric Gottenberg, A. A. Kruize, P. Sandhya, S. Gandolfo, Seung-Ki Kwok, M. Kvarnstrom, R. Solans, D. Sene, Y. Suzuki, D. A. Isenberg, V. Valim, B. Hofauer, R. Giacomelli, V. Devauchelle-Pensec, F. Atzeni, T. A. Gheita, J. Morel, R. Izzo, U. Kalyoncu, A. Szántó, P. Olsson, H. Bootsma, M. Ramos-Casals, B. Kostov, P. Brito-Zerón, Sjögren Big Data Consortium
Dátum:2020
ISSN:0392-856X
Megjegyzések:Objectives: To evaluate the systemic phenotype associated with the presence of isolated anti-La/SSB antibodies in a large international registry of patients with primary Sjögren's syndrome (pSS) fulfilling the 2002 classification criteria. Methods: The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. Baseline clinical information from leading centres on clinical research in SS of the 5 continents was collected. Combination patterns of anti-Ro/SSA-La/SSB antibodies at the time of diagnosis defined the following four immunological phenotypes: double positive (combined Ro/SSA and La/SSB,) isolated anti-Ro/SSA, isolated anti-La/SSB, and immunonegative. Results: The cohort included 12,084 patients (11,293 females, mean 52.4 years) with recorded ESSDAI scores available. Among them, 279 (2.3%) had isolated anti-La/SSB antibodies. The mean total ESSDAI score at diagnosis of patients with pSS carrying isolated anti-La/SSB was 6.0, and 80.4% of patients had systemic activity (global ESSDAI score ?1) at diagnosis. The domains with the highest frequency of active patients were the biological (42.8%), glandular (36.8%) and articular (31.2%) domains. Patients with isolated anti-La/SSB showed a higher frequency of active patients in all ESSDAI domains but two (articular and peripheral nerve) in comparison with immune-negative patients, and even a higher absolute frequency in six clinical ESSDAI domains in comparison with patients with isolated anti-Ro/SSA. In addition, patients with isolated anti-La/SSB showed a higher frequency of active patients in two ESSDAI domains (pulmonary and glandular) with respect to the most active immunological subset (double-positive antibodies). Meanwhile, systemic activity detected in patients with isolated anti-La/SSB was overwhelmingly low. Even in ESSDAI domains where patients with isolated anti-La/SSB had the highest frequencies of systemic activity (lymphadenopathy and muscular), the percentage of patients with moderate or high activity was lower in comparison with the combined Ro/SSA and La/SSB group. Conclusions: Patients carrying isolated La/SSB antibodies represent a very small subset of patients with a systemic SS phenotype characterised by a significant frequency of active patients in most clinical ESSDAI domains but with a relative low frequency of the highest severe organ-specific involvements. Primary SS still remains the best clinical diagnosis for this subset of patients.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
primary Sjögren's syndrome
isolated La/SSB autoantibodies
anti-Ro/SSA antibodies
systemic disease
ESSDAI
big data
Megjelenés:Clinical and Experimental Rheumatology. - 38 : 4 (2020), p. 85-94. -
További szerzők:Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Mandl, Thomas Priori, Roberta Vissink, Arjan Hernandez-Molina, Gabriela Armagan, Berkan Praprotnik, Sonja Sebastian, Agata Bartoloni, Elena Rischmueller, Maureen Pasoto, Sandra Nordmark, Gunnel Nakamura, Hideki Fernandes Moça Trevisani, Virginia Retamozo, Soledad Carsons, Steven E. Maure-Noia, B. Sánchez-Berná, I. López-Dupla, Miguel Fonseca-Aizpuru, Eva Melchor Díaz, S. Vázquez, Marta Díaz Cuiza, P. E. Miguel Campo, B. de Ng, Wan Fai Rasmussen, Astrid Dong, X. Li, X. Baldini, Chiara Seror, Raphaele Gottenberg, Jacques-Eric Kruize, Aike A. Sandhya, Pulukool Gandolfo, Saviana Kwok, Seung-Ki Kvarnstrom, Marika Solans, Roser Sene, Damien Suzuki, Yasunori Isenberg, David A. Valim, Valeria Hofauer, Benedikt Giacomelli, Roberto Devauchelle-Pensec, Valerie Atzeni, F. Gheita, Tamer A. Morel, Jacques Izzo, R. Kalyoncu, U. Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Olsson, Peter Bootsma, Hendrika Ramos-Casals, Manuel Kostov, Belchin Brito-Zerón, Pilar Sjögren Big Data Consortium
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2.

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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3.

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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4.

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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5.

001-es BibID:BIBFORM083560
035-os BibID:(WOS)000574394600030 (Scopus)85078897832
Első szerző:Brito-Zerón, Pilar
Cím:Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjögren's syndrome / Pilar Brito-Zerón, Nihan Acar-Denizli, Wan-Fai Ng, Ildiko Fanny Horváth, Astrid Rasmussen, Raphaele Seror, Xiaomei Li, Chiara Baldini, Jacques-Eric Gottenberg, Debashish Danda, Luca Quartuccio, Roberta Priori, Gabriela Hernandez-Molina, Berkan Armagan, Aike A. Kruize, Seung-Ki Kwok, Marika Kvarnstrom, Sonja Praprotnik, Damien Sene, Roberto Gerli, Roser Solans, Maureen Rischmueller, Thomas Mandl, Yasunori Suzuki, David Isenberg, Valeria Valim, Piotr Wiland, Gunnel Nordmark, Guadalupe Fraile, Hendrika Bootsma, Hideki Nakamura, Roberto Giacomelli, Valerie Devauchelle-Pensec, Benedikt Hofauer, Michele Bombardieri, Virginia Fernandes Moça Trevisani, Daniel Hammenfors, Sandra G. Pasoto, Soledad Retamozo, Tamer A. Gheita, Fabiola Atzeni, Jacques Morel, Cristina Vollenweider, Margit Zeher, Kathy Sivils, Bei Xu, Stefano Bombardieri, Pulukool Sandhya, Salvatore De Vita, Antonina Minniti, Jorge Sánchez-Guerrero, Levent Kilic, Eefje van der Heijden, Sung-Hwan Park, Marie Wahren-Herlenius, Xavier Mariette, Manuel Ramos-Casals, Sjögren Big Data Consortium
Dátum:2020
ISSN:1462-0324 1462-0332
Megjegyzések:OBJECTIVE: To characterize the systemic phenotype of primary Sjögren's syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. METHODS: The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren's syndrome from the five continents. RESULTS: The cohort included 10 007 patients (9352 female, mean 53?years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ?1). Males had a higher mean ESSDAI (8.1 vs 6.0, P?<?0.001) compared with females, as did patients diagnosed at <35?years (6.7 vs 5.6 in patients diagnosed at >65?years, P?<?0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P?<?0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P?<?0.001). CONCLUSION: The systemic phenotype of primary Sjögren's syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
ethnicity
gender
geoepidemiology
phenotype
primary Sjögren's syndrome
Megjelenés:Rheumatology. - 59 : 9 (2020), p. 2350-2359. -
További szerzők:Acar-Denizli, Nihan Ng, Wan Fai Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Rasmussen, Astrid Seror, Raphaele Li, Xiaomei Baldini, Chiara Gottenberg, Jacques-Eric Danda, Debashish Quartuccio, Luca Priori, Roberta Hernandez-Molina, Gabriela Armagan, Berkan Kruize, Aike A. Kwok, Seung-Ki Kvarnstrom, Marika Praprotnik, Sonja Sene, Damien Gerli, Roberto Solans, Roser Rischmueller, Maureen Mandl, Thomas Suzuki, Yasunori Isenberg, David A. Valim, Valeria Wiland, Piotr Nordmark, Gunnel Fraile, Guadalupe Bootsma, Hendrika Nakamura, Hideki Giacomelli, Roberto Devauchelle-Pensec, Valerie Hofauer, Benedikt Bombardieri, Michele Trevisani, Virginia Fernandes Moça Hammenfors, Daniel Pasoto, Sandra Retamozo, Soledad Gheita, Tamer A. Atzeni, Fabiola Morel, Jacques Vollenveider, Cristina Zeher Margit (1957-2018) (belgyógyász, allergológus és klinikai immunológus, reumatológus) Sivils, Kathy Xu, Bei Bombardieri, Stefano Sandhya, Pulukool Vita, Salvatore de Minniti, Antonina Sanchez-Guerrero, Jorge Kilic, Levent Heijden, Eefje van der Park, Sung-Hwan Wahren-Herlenius, Marie Mariette, Xavier Ramos-Casals, Manuel Sjögren Big Data Consortium
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6.

001-es BibID:BIBFORM074743
035-os BibID:(WOS)000446486100015 (Scopus)85055613559
Első szerző:Brito-Zerón, Pilar
Cím:How immunological profile drives clinical phenotype of primary Sjögren's syndrome at diagnosis : analysis of 10,500 patients (Sjögren Big Data Project) / P. Brito-Zerón, N. Acar-Denizli, W. F. Ng, M. Zeher, A. Rasmussen, T. Mandl, R. Seror, X. Li, C. Baldini, Jacques-Eric Gottenberg, D. Danda, L. Quartuccio, R. Priori, G. Hernandez-Molina, B. Armagan, A. A. Kruize, Seung-Ki Kwok, M. Kvarnström, S. Praprotnik, D. Sène, E. Bartoloni, R. Solans, M. Rischmueller, Y. Suzuki, D. A. Isenberg, V. Valim, P. Wiland, G. Nordmark, G. Fraile, H. Bootsma, T. Nakamura, R. Giacomelli, V. Devauchelle-Pensec, A. Knopf, M. Bombardieri, V. Trevisani, D. Hammenfors, S. Pasoto, S. Retamozo, T. A. Gheita, F. Atzeni, J. Morel, C. Vollenveider, I. Horvath, K. Sivils, P. Olsson, S. De Vita, J. Sánchez-Guerrero, L. Kilic, M. Wahren-Herlenius, X. Mariette, M. Ramos-Casals, Sjögren Big Data Consortium
Dátum:2018
ISSN:0392-856X
Megjegyzések:OBJECTIVES:To evaluate the influence of the main immunological markers on the disease phenotype at diagnosis in a large international cohort of patients with primary Sjögren's syndrome (SjS). METHODS:The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. As a first step, baseline clinical information from leading centres on clinical research in SjS of the 5 continents was collected. The centres shared a harmonised data architecture and conducted cooperative online efforts in order to refine collected data under the coordination of a big data statistical team. Inclusion criteria were the fulfillment of the 2002 classification criteria. Immunological tests were carried out using standard commercial assays. RESULTS:By January 2018, the participant centres had included 10,500 valid patients from 22 countries. The cohort included 9,806 (93%) women and 694 (7%) men, with a mean age at diagnosis of primary SjS of 53 years, mainly White (78%) and included from European countries (71%). The frequency of positive immunological markers at diagnosis was 79.3% for ANA, 73.2% for anti-Ro, 48.6% for RF, 45.1% for anti- La, 13.4% for low C3 levels, 14.5% for low C4 levels and 7.3% for cryoglobulins. Positive autoantibodies (ANA, Ro, La) correlated with a positive result in salivary gland biopsy, while hypocomplementaemia and especially cryoglo-bulinaemia correlated with systemic activity (mean ESSDAI score of 17.7 for cryoglobulins, 11.3 for low C3 and 9.2 for low C4, in comparison with 3.8 for negative markers). The immunological markers with a great number of statistically-significant associations (p<0.001) in the organ-by-organ ESS- DAI evaluation were cryoglobulins (9 domains), low C3 (8 domains), anti-La (7 domains) and low C4 (6 domains). CONCLUSIONS:We confirm the strong influence of immunological markers on the phenotype of primary SjS at diagnosis in the largest multi-ethnic international cohort ever analysed, with a greater influence for cryoglobulinaemic-related markers in comparison with Ro/La autoantibodies and ANA. Immunological patterns play a central role in the phenotypic expression of the disease already at the time of diagnosis, and may guide physicians to design a specific personalised management during the follow-up of patients with primary SjS.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
primary Sjögren's syndrome
salivary gland biopsy
Ro/La autoantibodies
hypocomplementaemia
cryoglobulinaemia
ESSDAI
Megjelenés:Clinical and Experimental Rheumatology. - 36 : Suppl. 112 (2018), p. S102-S112. -
További szerzők:Acar-Denizli, Nihan Ng, Wan Fai Zeher Margit (1957-2018) (belgyógyász, allergológus és klinikai immunológus, reumatológus) Rasmussen, Astrid Mandl, Thomas Seror, Raphaele Li, X. Baldini, Chiara Gottenberg, Jacques-Eric Danda, Debashish Quartuccio, Luca Priori, Roberta Hernandez-Molina, Gabriela Armagan, Berkan Kruize, Aike A. Kwok, Seung-Ki Kvarnstrom, Marika Praprotnik, Sonja Sene, Damien Bartoloni, Elena Solans, Roser Rischmueller, Maureen Suzuki, Yasunori Isenberg, David A. Valim, Valeria Wiland, Piotr Nordmark, Gunnel Fraile, Guadalupe Bootsma, Hendrika Nakamura, Tomohiro Giacomelli, Roberto Devauchelle-Pensec, Valerie Knopf, A. Bombardieri, Michele Trevisani, Virginia Fernandes Moça Hammenfors, Daniel Pasoto, Sandra Retamozo, Soledad Gheita, Tamer A. Atzeni, F. Morel, Jacques Vollenveider, Cristina Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Sivils, Kathy Olsson, Peter Vita, Salvatore de Sanchez-Guerrero, Jorge Kilic, Levent Wahren-Herlenius, Marie Mariette, Xavier Ramos-Casals, Manuel Sjögren Big Data Consortium
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7.

001-es BibID:BIBFORM069228
035-os BibID:(WoS)000401138800017 (Scopus)85006013155
Első szerző:Brito-Zerón, Pilar
Cím:Influence of geolocation and ethnicity on the phenotypic expression of primary Sjögren's syndrome at diagnosis in 8310 patients : a cross-sectional study from the Big Data Sjögren Project Consortium / Pilar Brito-Zerón, Nihan Acar-Denizli, Margit Zeher, Astrid Rasmussen, Raphaele Seror, Elke Theander, Xiaomei Li, Chiara Baldini, Jacques-Eric Gottenberg, Debashish Danda, Luca Quartuccio, Roberta Priori, Gabriela Hernandez-Molina, Aike A. Kruize, Valeria Valim, Marika Kvarnstrom, Damien Sene, Roberto Gerli, Sonja Praprotnik, David Isenberg, Roser Solans, Maureen Rischmueller, Seung-Ki Kwok, Gunnel Nordmark, Yasunori Suzuki, Roberto Giacomelli, Valerie Devauchelle-Pensec, Michele Bombardieri, Benedikt Hofauer, Hendrika Bootsma, Johan G. Brun, Guadalupe Fraile, Steven E. Carsons, Tamer A. Gheita, Jacques Morel, Cristina Vollenveider, Fabiola Atzeni, Soledad Retamozo, Ildiko Fanny Horvath, Kathy Sivils, Thomas Mandl, Pulukool Sandhya, Salvatore De Vita, Jorge Sanchez-Guerrero, Eefje van der Heijden, Virginia Fernandes Moça Trevisani, Marie Wahren-Herlenius, Xavier Mariette, Manuel Ramos-Casals, EULAR-SS Task Force Big Data Consortium
Dátum:2017
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Annals of the rheumatic diseases. - 76 : 6 (2017), p. 1042-1050. -
További szerzők:Acar-Denizli, Nihan Zeher Margit (1957-2018) (belgyógyász, allergológus és klinikai immunológus, reumatológus) Rasmussen, Astrid Seror, Raphaele Theander, Elke Li, Xiaomei Baldini, Chiara Gottenberg, Jacques-Eric Danda, Debashish Quartuccio, Luca Priori, Roberta Hernandez-Molina, Gabriela Kruize, Aike A. Valim, Valeria Kvarnstrom, Marika Sene, Damien Gerli, Roberto Praprotnik, Sonja Isenberg, David A. Solans, Roser Rischmueller, Maureen Kwok, Seung-Ki Nordmark, Gunnel Suzuki, Yasunori Giacomelli, Roberto Devauchelle-Pensec, Valerie Bombardieri, Michele Hofauer, Benedikt Bootsma, Hendrika Brun, Johan G. Fraile, Guadalupe Carsons, Steven E. Gheita, Tamer A. Morel, Jacques Vollenveider, Cristina Atzeni, Fabiola Retamozo, Soledad Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Sivils, Kathy Mandl, Thomas Sandhya, Pulukool Vita, Salvatore de Sanchez-Guerrero, Jorge Heijden, Eefje van der Fernandes Moça Trevisani, Virginia Wahren-Herlenius, Marie Mariette, Xavier Ramos-Casals, Manuel EULAR-SS Task Force Big Data Consortium
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8.

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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DOI
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9.

001-es BibID:BIBFORM083561
035-os BibID:(WOS)000488952900016 (Scopus)85071621125
Első szerző:Retamozo, Soledad
Cím:Systemic manifestations of primary Sjögren's syndrome out of the ESSDAI classification : prevalence and clinical relevance in a large international, multi-ethnic cohort of patients / S. Retamozo, N. Acar-Denizli, A. Rasmussen, I. F. Horváth, C. Baldini, R. Priori, P. Sandhya, G. Hernandez-Molina, B. Armagan, S. Praprotnik, M. Kvarnstrom, R. Gerli, A. Sebastian, R. Solans, M. Rischmueller, S. G. Pasoto, V. Valim, G. Nordmark, A. A. Kruize, H. Nakamura, B. Hofauer, R. Giacomelli, V. Fernandes Moça Trevisani, V. Devauchelle-Pensec, F. Atzeni, T. A. Gheita, S. Consani-Fernández, A. Szántó, K. Sivils, A. Gattamelata, D. Danda, L. Kilic, E. Bartoloni, S. Bombardieri, J. Sánchez-Guerrero, M. Wahren-Herlenius, X. Mariette, M. Ramos-Casals, P. Brito-Zerón, Sjögren Big Data Consortium
Dátum:2019
ISSN:0392-856X
Megjegyzések:OBJECTIVES: To analyse the frequency and characterise the systemic presentation of primary Sjögren's syndrome (SS) out of the ESSDAI classification in a large international, multi-ethnic cohort of patients. METHODS: The Big Data Sjögren Project Consortium is an international, multicentre registry based on world-wide data-sharing and cooperative merging of pre-existing clinical SS databases from leading centres in clinical research in SS from the five continents. A list of 26 organ-by-organ systemic features not currently included in the ESSDAI classification was defined according to previous studies; these features were retrospectively recorded. RESULTS: Information about non-ESSDAI features was available in 6331 patients [5,917 female, mean age at diagnosis 52 years, mainly White (86.3%)]. A total of 1641 (26%) patients had at least one of the ESSDAI systemic features. Cardiovascular manifestations were the most frequent organ-specific group of non-ESSDAI features reported in our patients (17% of the total cohort), with Raynaud's phenomenon being reported in 15%. Patients with systemic disease due to non-ESSDAI features had a lower frequency of dry mouth (90.7% vs. 94.1%, p<0.001) and positive minor salivary gland biopsy (86.7% vs. 89%, p=0.033), a higher frequency of anti-Ro/SSA (74.7% vs. 68.7%, p<0.001), anti-La/SSB antibodies (44.5% vs. 40.4%, p=0.004), ANA (82.7% vs. 79.5%, p=0.006), low C3 levels (17.4% vs. 9.7%, p<0.001), low C4 levels (14.4% vs. 9.6%, p<0.001), and positive serum cryoglobulins (8.6% vs. 5.5%, p=0.001). Systemic activity measured by the ESSDAI, clinESSDAI and DAS was higher in patients with systemic disease out of the ESSDAI in comparison with those without these features (p<0.001 for all comparisons). CONCLUSIONS: More than a quarter of patients with primary SS may have systemic manifestations not currently included in the ESSDAI classification, with a wide variety of cardiovascular, digestive, pulmonary, neurological, ocular, ENT (ear, nose, and throat), cutaneous and urological features that increase the scope of the systemic phenotype of the disease. However, the individual frequency of each of these non-ESSDAI features was very low, except for Raynaud's phenomenon.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
primary Sjögren's syndrome
ESSDAI
Raynaud's phenomenon
pleuritis
pericarditis
uveitis
congenital heart block
pulmonary arterial hypertension
Megjelenés:Clinical and Experimental Rheumatology. - 37 Suppl. 118 : 3 (2019), p. 97-106. -
További szerzők:Acar-Denizli, Nihan Rasmussen, Astrid Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Baldini, Chiara Priori, Roberta Sandhya, Pulukool Hernandez-Molina, Gabriela Armagan, Berkan Praprotnik, Sonja Kvarnstrom, Marika Gerli, Roberto Sebastian, Agata Solans, Roser Rischmueller, Maureen Pasoto, Sandra Valim, Valeria Nordmark, Gunnel Kruize, Aike A. Nakamura, Hideki Hofauer, Benedikt Giacomelli, Roberto Fernandes Moça Trevisani, Virginia Devauchelle-Pensec, Valerie Atzeni, F. Gheita, Tamer A. Consani-Fernández, S. Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Sivils, Kathy Gattamelata, Angelica Danda, Debashish Kilic, Levent Bartoloni, Elena Bombardieri, Stefano Sanchez-Guerrero, Jorge Wahren-Herlenius, Marie Mariette, Xavier Ramos-Casals, Manuel Brito-Zerón, Pilar Sjögren Big Data Consortium
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