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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:BIBFORM120110
035-os BibID:(WoS)001134013500010 (Scopus)85181176271
Első szerző:Brito-Zerón, Pilar
Cím:Exposure to air pollution as an environmental determinant of how Sjögren's disease is expressed at diagnosis / Brito-Zerón Pilar, Flores-Chávez Alejandra, Ng Wan-Fai, Fanny Horváth Ildiko, Rasmussen Astrid, Priori Roberta, Baldini Chiara, Armagan Berkan, Özkiziltas Burcugül, Praprotnik Sonja, Suzuki Yasunori, Quartuccio Luca, Hernandez-Molina Gabriela, Abacar Kerem, Bartoloni Elena, Rischmueller Maureen, Reis-de Oliveira Fabiola, Fernandes Moca Trevisani Virginia, Jurcut Ciprian, Fugmann Cecilia, Carubbi Francesco, Hofauer Benedikt, Valim Valeria, Pasoto Sandra G., Retamozo Soledad, Atzeni Fabiola, Fonseca-Aizpuru Eva, López-Dupla Miguel, Giacomelli Roberto, Nakamura Hideki, Akasbi Miriam, Thompson Kyle, Szántó Antónia, Farris A. Darise, Villa Martina, Bombardieri Stefano, Kilic Levent, Tufan Abdurrahman, Perdan Pirkmajer Katja, Fujisawa Yuhei, de Vita Salvatore, Inanc Nevsun, Ramos-Casals Manuel, Sjögren Big Data Consortium
Dátum:2023
ISSN:0392-856X 1593-098X
Megjegyzések:Objectives: To analyse how the potential exposure to air pollutants can influence the key components at the time of diagnosis of Sjögren's phenotype (epidemiological profile, sicca symptoms, and systemic disease). Methods: For the present study, the following variables were selected for harmonization and refinement: age, sex, country, fulfilment of 2002/2016 criteria items, dry eyes, dry mouth, and overall ESSDAI score. Air pollution indexes per country were defined according to the OECD (1990-2021), including emission data of nitrogen and sulphur oxides (NO/SO), particulate matter (PM2.5 and 1.0), carbon monoxide (CO) and volatile organic compounds (VOC) calculated per unit of GDP, Kg per 1000 USD. Results: The results of the chi-square tests of independence for each air pollutant with the frequency of dry eyes at diagnosis showed that, except for one, all variables exhibited p-values <0.0001. The most pronounced disparities emerged in the dry eye prevalence among individuals inhabiting countries with the highest NO/SO exposure, a surge of 4.61 percentage points compared to other countries, followed by CO (3.59 points), non-methane (3.32 points), PM2.5 (3.30 points), and PM1.0 (1.60 points) exposures. Concerning dry mouth, individuals residing in countries with worse NO/SO exposures exhibited a heightened frequency of dry mouth by 2.05 percentage points (p<0.0001), followed by non-methane exposure (1.21 percentage points increase, p=0.007). Individuals inhabiting countries with the worst NO/SO, CO, and PM2.5 pollution levels had a higher mean global ESSDAI score than those in lower-risk nations (all p-values <0.0001). When systemic disease was stratified according to DAS into low, moderate, and high systemic activity levels, a heightened proportion of individuals manifesting moderate/severe systemic activity was observed in countries with worse exposures to NO/SO, CO, and PM2.5 pollutant levels. Conclusions: For the first time, we suggest that pollution levels could influence how SjD appears at diagnosis in a large international cohort of patients. The most notable relationships were found between symptoms (dryness and general body symptoms) and NO/SO, CO, and PM2.5 levels.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's syndrome
dryness
systemic
ESSDAI
air pollution
environment
Megjelenés:Clinical And Experimental Rheumatology. - 41 : 12 (2023), p. 2448-2457. -
További szerzők:Flores-Chávez, Alejandra Ng, Wan Fai Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Rasmussen, Astrid Priori, Roberta Baldini, Chiara Armagan, Berkan Özkiziltaș, Burcugül Praprotnik, Sonja Suzuki, Yasunori Quartuccio, Luca Hernandez-Molina, Gabriela Abacar, Kerem Bartoloni, Elena Rischmueller, Maureen Reis-de Oliveira, Fabiola Fernandes Moça Trevisani, Virginia Jurcut, Ciprian Fugmann, Cecilia Carubbi, Francesco Hofauer, Benedikt Valim, Valeria Pasoto, Sandra Retamozo, Soledad Atzeni, Fabiola Fonseca-Aizpuru, Eva López-Dupla, Miguel Giacomelli, Roberto Nakamura, Hideki Akasbi, Miriam Thompson, Kyle Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Farris, Darise Villa, Martina Bombardieri, Stefano Kilic, Levent Tufan, Abdurrahman Perdan Pirkmajer, Katja Fujisawa, Yuhei Vita, Salvatore de Inanc, Nevsun 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: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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6.

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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7.

001-es BibID:BIBFORM091877
Első szerző:Fisher, Benjamin
Cím:Assessment of the anti-CD40 antibody iscalimab in patients with primary Sjögren's syndrome : a multicentre, randomised, double-blind, placebo-controlled, proof-of-concept study / Benjamin A. Fisher, Antonia Szanto, Wan-Fai Ng, Michele Bombardieri, Maximilian G. Posch, Athena S. Papas, Arwa M. Farag, Thomas Daikeler, Bettina Bannert, Diego Kyburz, Alan J. Kivitz, Steven E. Carsons, David A. Isenberg, Francesca Barone, Simon J. Bowman, Pascal Espié, David Floch, Cyrielle Dupuy, Xiaohui Ren, Petra M. Faerber, Andrew M. Wright, Hans-Ulrich Hockey, Michael Rotte, Julie Milojevic, Alexandre Avrameas, Marie-Anne Valentin, James S. Rush, Peter Gergely
Dátum:2020
ISSN:2665-9913
Megjegyzések:Background Primary Sjogren's syndrome is an autoimmune disease that presents as dryness of the mouth and eyes due to impairment of the exocrine glands. To our knowledge, no systemic therapies for primary Sjogren's syndrome have shown efficacy. CD40-CD154-mediated T cell-B cell interactions in primary Sjogren's syndrome contribute to aberrant lymphocyte activation in inflamed tissue, leading to sialadenitis and other tissue injury. Therefore, we investigated the safety and preliminary efficacy of iscalimab (CFZ533), a novel anti-CD40 monoclonal antibody, in patients with primary Sjogren's syndrome. Methods This multicentre, randomised, double-blind, placebo-controlled, proof-of-concept study took place at ten investigational sites across Europe (UK, n=4; Germany, Switzerland, and Hungary, n=1 each) and the USA (n=3). Eligible patients were aged 18-75 years and fulfilled the 2002 American European consensus group diagnostic dassification criteria for primary Sjogren's syndrome. In the double-blind phase of the trial, patients were randomly assigned (2:1) via computer-generated unique randomisation numbers to receive subcutaneous iscalimab (3 mg/kg) or placebo at weeks 0, 2, 4, and 8 (cohort 1) or intravenous iscalimab (10 mg/kg) or placebo at weeks 0, 2, 4, and 8 (cohort 2). Randomisation was stratified according to baseline intake of oral corticosteroids. At week 12, patients in both cohorts received open-label iscalimab (same dose and route) for 12 weeks. The primary objectives of the study were to assess the safety, tolerability, and efficacy of multiple doses of iscalimab in the two sequential dose cohorts. Safety and tolerability were assessed by adverse events and efficacy of iscalimab versus placebo was assessed by dinical disease activity, as measured by the change in European League Against Rheumatism Sjogren's syndrome disease activity index (ESSDAI) score after 12 weeks of treatment. Analyses were done on a per-protocol basis. The trial was registered with ClinicalTrials.gov, NCT02291029. Findings Between Oct 22, 2014, and June 28, 2016, we assessed 82 patients for eligibility (25 for cohort 1 and 57 for cohort 2). 38 patients were excluded because of ineligibility. In cohort 1, 12 patients were randomly assigned to receive either 3 mg/kg doses of iscalimab (n=8) or placebo (n=4), and in cohort 2, 32 patients were randomly assigned to receive either intravenous 10 mg/kg doses of iscalimab (n=21) or placebo (n=11). Adverse events were similar between iscalimab treatment groups and placebo groups, with adverse events occurring in all patients in cohort 1, and in 52% and 64% of the iscalimab and placebo groups, respectively, in cohort 2. Two serious adverse events were reported (one case of bacterial conjunctivitis in cohort 1 and one case of atrial fibrillation in cohort 2), which were unrelated to treatment with iscalimab. Intravenous treatment with iscalimab resulted in a mean reduction of 5.21 points (95% CI 0.96-9.46; one-sided p=0.0090) in ESSDAI score compared with placebo. There was no signficiant difference in ESSDAI score between subcutaneous iscalimab and placebo. Interpretation To our knowledge, this is the first randomised, placebo-controlled proof-of-concept study of a new investigational drug for primary Sjogren's syndrome that indicates preliminary efficacy. Our data suggest a role of CD40-CD154 interactions in primary Sjogren's syndrome pathology and the therapeutic potential for CD40 blockade in this disease should be investigated further. Copyright (C) 2020 Elsevier Ltd. All rights reserved
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
GERMINAL CENTER FORMATION
EPITHELIAL-CELLS
DISEASE-ACTIVITY
EXPRESSION
CD40
APOPTOSIS
CXCL13
Megjelenés:The Lancet Rheumatology. - 2 : 3 (2020), p. 142-152. -
További szerzők:Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Ng, Wan Fai Bombardieri, Michele Posch, Maximilian G. Papas, Athena S. Farag, Arwa M. Daikeler, Thomas Bannert, Bettina Kyburz, Diego Kivitz, Alan J. Carsons, Steven E. Isenberg, David A. Barone, Francesca Bowman, Simon J. Espié, Pascal Floch, David Dupuy, Cyrielle Ren, Xiaohui Faerber, Petra M. Wright, Andrew M. Hockey, Hans-Ulrich Rotte, Michael Milojevic, Julie Avrameas, Alexandre Valentin, Marie-Anne Rush, James S. Gergely Péter (Budapest)
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Borító:

8.

001-es BibID:BIBFORM119113
035-os BibID:(scopus)85181178127 (WoS)001134013500012
Első szerző:Flores-Chávez, Alejandra
Cím:Influence of exposure to climate-related hazards in the phenotypic expression of primary Sjögren's syndrome / Flores-Chávez Alejandra, Brito-Zerón Pilar, Ng Wan-Fai, Szántó Antónia, Rasmussen Astrid, Priori Roberta, Baldini Chiara, Armagan Berkan, Özkiziltas Burcugül, Praprotnik Sonja, Suzuki Yasunori, Quartuccio Luca, Hernández-Molina Gabriela, Inanc Nevsun, Bartoloni Elena, Rischmueller Maureen, Reis-de Oliveira Fabiola, Fernandes Moca Trevisani Virginia, Jurcut Ciprian, Nordmark Gunnel, Carubbi Francesco, Hofauer Benedikt, Valim Valeria, Pasoto Sandra G., Retamozo Soledad, Atzeni Fabiola, Fonseca-Aizpuru Eva, López-Dupla Miguel, Giacomelli Roberto, Nakamura Hideki, Akasbi Miriam, Thompson Kyle, Fanny Horváth Ildiko, Farris A. Darise, Simoncelli Edoardo, Bombardieri Stefano, Kilic Levent, Tufan Abdurrahman, Perdan Pirkmajer Katja, Fujisawa Yuhei, De Vita Salvatore, Abacar Kerem, Ramos-Casals Manuel, Sjögren Big Data Consortium
Dátum:2023
ISSN:0392-856X 1593-098X
Megjegyzések:OBJECTIVES: To analyse how the key components at the time of diagnosis of the Sjögren's phenotype (epidemiological profile, sicca symptoms, and systemic disease) can be influenced by the potential exposure to climate-related natural hazards. METHODS: For the present study, the following variables were selected for harmonisation and refinement: age, sex, country, fulfilment of 2002/2016 criteria items, dry eyes, dry mouth, and overall ESSDAI score. Climate-related hazards per country were defined according to the OECD and included seven climate-related hazard types: extreme temperature, extreme precipitation, drought, wildfire, wind threats, river flooding, and coastal flooding. Climatic variables were defined as dichotomous variables according to whether each country is ranked among the ten countries with the most significant exposure. RESULTS: After applying data-cleaning techniques and excluding people from countries not included in the OECD climate rankings, the database study analysed 16,042 patients from 23 countries. The disease was diagnosed between 1 and 3 years earlier in people living in countries included among the top 10 worst exposed to extreme precipitation, wildfire, wind threats, river flooding, and coastal flooding. A lower frequency of dry eyes was observed in people living in countries exposed to wind threats, river flooding, and coastal flooding, with a level of statistical association being classified as strong (p<0.0001 for the three variables). The frequency of dry mouth was significantly lower in people living in countries exposed to river flooding (p<0.0001) and coastal flooding (p<0.0001). People living in countries included in the worse climate scenarios for extreme temperature (p<0.0001) and river flooding (p<0.0001) showed a higher mean ESSDAI score in comparison with people living in no-risk countries. In contrast, those living in countries exposed to worse climate scenarios for wind threats (p<0.0001) and coastal flooding (p<0.0001) showed a lower mean ESSDAI score in comparison with people living in no-risk countries. CONCLUSIONS: Local exposure to extreme climate-related hazards plays a role in modulating the presentation of Sjögren across countries concerning the age at which the disease is diagnosed, the frequency of dryness, and the degree of systemic activity.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's syndrome
dryness
systemic
ESSDAI
climate
epidemiology
Megjelenés:Clinical And Experimental Rheumatology. - 41 : 12 (2023), p. 2437-2447. -
További szerzők:Brito-Zerón, Pilar Ng, Wan Fai Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Rasmussen, Astrid Priori, Roberta Baldini, Chiara Armagan, Berkan Özkiziltaș, Burcugül Praprotnik, Sonja Suzuki, Yasunori Quartuccio, Luca Hernandez-Molina, Gabriela Inanc, Nevsun Bartoloni, Elena Rischmueller, Maureen Reis-de Oliveira, Fabiola Fernandes Moça Trevisani, Virginia Jurcut, Ciprian Nordmark, Gunnel Carubbi, Francesco Hofauer, Benedikt Valim, Valeria Pasoto, Sandra Retamozo, Soledad Atzeni, Fabiola Fonseca-Aizpuru, Eva López-Dupla, Miguel Giacomelli, Roberto Nakamura, Hideki Akasbi, Miriam Thompson, Kyle Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Farris, Darise Simoncelli, Edoardo Bombardieri, Stefano Kilic, Levent Tufan, Abdurrahman Perdan Pirkmajer, Katja Fujisawa, Yuhei Vita, Salvatore de Abacar, Kerem Ramos-Casals, Manuel Sjögren Big Data Consortium
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Intézményi repozitóriumban (DEA) tárolt változat
Borító:

9.

001-es BibID:BIBFORM104986
035-os BibID:(WOS)000786680100001 (Scopus)85139097101
Első szerző:Hernandez-Molina, Gabriela
Cím:Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies / Hernández-Molina Gabriela, Kostov Belchin, Brito-Zerón Pilar, Vissink Arjan, Mandl Thomas, Hinrichs Anneline C., Quartuccio Luca, Baldini Chiara, Seror Raphaele, Szántó Antonia, Isenberg David, Gerli Roberto, Nordmark Gunnel, Rasmussen Astrid, Solans-Laque Roser, Hofauer Benedikt, Sene Damien, Pasoto Sandra G., Rischmueller Maureen, Praprotnik Sonja, Gheita Tamer A., Danda Debashish, Armagan Berkan, Suzuki Yasunori, Valim Valeria, Devauchelle-Pensec Valerie, Retamozo Soledad, Kvarnstrom Marika, Sebastian Agata, Atzeni Fabiola, Giacomelli Roberto, Carsons Steven E., Kwok Seung-Ki, Nakamura Hideki, Fernandes Moca Trevisani Virginia, Flores-Chávez Alejandra, Mariette Xavier, Ramos-Casals Manuel, Sjögren Big Data Consortium
Dátum:2023
ISSN:1462-0324 1462-0332
Megjegyzések:Abstract Objective To characterize 414 patients with primary SS who developed haematological malignancies and to analyse how the main SS- and lymphoma-related features can modify the presentation patterns and outcomes. Methods By January 2021, the Big Data Sjögren Project Consortium database included 11?966 patients fulfilling the 2002/2016 classification criteria. Haematological malignancies diagnosed according to the World Health Organization (WHO) classification were retrospectively identified. Results There were 414 patients (355 women, mean age 57?years) with haematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). A total of 376 (91%) patients had mature B-cell malignancy, nearly half had extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT lymphoma) (n?=?197), followed by diffuse large B-cell lymphoma (DLBCL) (n?=?67), nodal MZL lymphoma (n?=?29), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) (n?=?19) and follicular lymphoma (FL) (n?=?17). Rates of complete response, relapses and death were 80%, 34% and 13%, respectively, with a 5-year survival rate of 86.5% after a mean follow-up of 8?years. There were significant differences in age at diagnosis (younger in MALT, older in CLL/SLL), predominant clinical presentation (glandular enlargement in MALT lymphoma, peripheral lymphadenopathy in nodal MZL and FL, constitutional symptoms in DLBCL, incidental diagnosis in CLL/SLL), therapeutic response (higher in MALT lymphoma, lower in DLBCL) and survival (better in MALT, nodal MZL and FL, worse in DLBCL). Conclusion In the largest reported study of haematological malignancies complicating primary SS, we confirm the overwhelming predominance of B-cell lymphomas, especially MALT, with the salivary glands being the primary site of involvement. This highly-specific histopathological scenario is linked with the overall good prognosis with a 5-year survival rate of nearly 90%.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's Syndrome
haematological malignancy
lymphoproliferative disease
lymphoma
MALT
Megjelenés:Rheumatology. - 62 : 1 (2023), p. 243-255. -
További szerzők:Kostov, Belchin Brito-Zerón, Pilar Vissink, Arjan Mandl, Thomas Hinrichs, Anneline Quartuccio, Luca Baldini, Chiara Seror, Raphaele Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Isenberg, David A. Gerli, Roberto Nordmark, Gunnel Rasmussen, Astrid Soláns-Laqué, Roser Hofauer, Benedikt Sene, Damien Pasoto, Sandra Rischmueller, Maureen Praprotnik, Sonja Gheita, Tamer A. Danda, Debashish Armagan, Berkan Suzuki, Yasunori Valim, Valeria Devauchelle-Pensec, Valerie Retamozo, Soledad Kvarnstrom, Marika Sebastian, Agata Atzeni, Fabiola Giacomelli, Roberto Carsons, Steven E. Kwok, Seung-Ki Nakamura, Hideki Fernandes Moça Trevisani, Virginia Flores-Chávez, Alejandra Mariette, Xavier Ramos-Casals, Manuel Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Tarr Tünde (1976-) (belgyógyász, allergológus és klinikai immunológus) Sjögren Big Data Consortium
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Borító:

10.

001-es BibID:BIBFORM105655
035-os BibID:(Scopus)85144589294
Első szerző:Inanc, Nevsun
Cím:Safety and efficacy of SARS-CoV-2 vaccination in 1237 patients with primary Sjögren syndrome / Inanc Nevsun, Kostov Belchin, Priori Roberta, Flores-Chavez Alejandra, Carubbi Francesco, Szántó Antónia, Valim Valeria, Bootsma Hendrika, Praprotnik Sonja, Fernandes Moca Trevisani Virginia, Hernández-Molina Gabriela, Hofauer Benedikt, Pasoto Sandra G., López-Dupla Miguel, Bartoloni Elena, Rischmueller Maureen, Devauchelle-Pensec Valerie, Abacar Kerem, Giardina Federico, Alunno Alessia, Fanny Horváth Ildikó, de Wolff Liseth, Caldas Laura, Retamozo Soledad, Ramos-Casals Manuel, Brito-Zerón Pilar, Sjögren Big Data Consortium
Dátum:2022
ISSN:0392-856X 1593-098X
Megjegyzések:OBJECTIVES: To investigate the safety and efficacy of SARS-Cov-2 vaccination in patients with primary Sjögren syndrome (pSS) due to scarcity of data in this population. METHODS: By the first week of May 2021, all Big Data SS Consortium centres patients who had received at least one dose of any SARS-CoV-2 vaccine were included in the study. The in-charge physician asked patients about local and systemic reactogenicity to collect SARS-CoV-2 vaccination data. RESULTS: The vaccination data of 1237 patients were received. A total of 835 patients (67%) reported any adverse events (AEs), including local (53%) and systemic (50%) AEs. Subjective symptoms (63%) were the most common local AEs, followed by objective signs at the injection site (16%), and general symptoms were the most commonly reported systemic AEs (46%), followed by musculoskeletal (25%), gastrointestinal (9%), cardiopulmonary (3%), and neurological (2%). In addition, 141 (11%) patients reported a significant worsening/exacerbation of their pre-vaccination sicca symptoms and fifteen (1.2%) patients reported active involvement in the glandular (n=7), articular (n=7), cutaneous (n=6), pulmonary (n=2), and peripheral nervous system (n=1) domains due to post-vaccination SS flares. In terms of vaccination efficacy, breakthrough SARS-CoV-2 infection was confirmed after vaccination in three (0.24 %) patients, and positive anti-SARS-Cov-2 antibodies were detected in approximately 95% of vaccinated SS patients, according to data available. CONCLUSIONS: Our data suggest that patients with pSS develop adequate humoral response and no severe AEs after SARS-CoV-2 vaccination and therefore raise no concerns about the vaccine's efficacy or safety profile in this population.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
primary Sjögren syndrome
SARS-Cov-2 vaccination
sjögren big data consortium
adverse events
disease flare
Megjelenés:Clinical And Experimental Rheumatology. - 40 : 12 (2022), p. 2290-2297. -
További szerzők:Kostov, Belchin Priori, Roberta Flores-Chávez, Alejandra Carubbi, Francesco Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Valim, Valeria Bootsma, Hendrika Praprotnik, Sonja Fernandes Moça Trevisani, Virginia Hernandez-Molina, Gabriela Hofauer, Benedikt Pasoto, Sandra López-Dupla, Miguel Bartoloni, Elena Rischmueller, Maureen Devauchelle-Pensec, Valerie Abacar, Kerem Giardina, Federico Alunno, Alessia Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) de Wolff, Liseth Caldas, Laura Retamozo, Soledad Ramos-Casals, Manuel Brito-Zerón, Pilar Sjögren Big Data Consortium
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Intézményi repozitóriumban (DEA) tárolt változat
Borító:

11.

001-es BibID:BIBFORM135729
035-os BibID:(scopus)105009928363 (wos)001505219700001
Első szerző:Ramos-Casals, Manuel
Cím:2023 International Rome consensus for the nomenclature of Sjögren disease / Ramos-Casals Manuel, Baer Alan N., Brito-Zerón María del Pilar, Hammitt Katherine M., Bouillot Coralie, Retamozo Soledad, Mackey Alison, Yarowsky David, Turner Breck, Blanck Jaime, Fisher Benjamin A., Akpek Esen K., Baldini Chiara, Bootsma Hendrika, Bowman Simon J., Dörner Thomas, Laing Leslie, Lieberman Scott M., Mariette Xavier, Pflugfelder Stephen C., Sankar Vidya, Sisó-Almirall Antoni, Tzioufas Athanasios G., Anaya Juan-Manuel, Armagan Berkan, Bombardieri Michele, Carsons Steven, de Vita Salvatore, Fox Robert I., Gerli Roberto, Giacomelli Roberto, Gottenberg Jacques-Eric, Hernández-Molina Gabriela, Jonsson Roland, Kruize Aike A., Kwok Seung-Ki, Li Xiaomei, McCoy Sara S., Ng Wan-Fai, Olsson Peter, Rischmueller Maureen, Saraux Alain, Scofield R. Hal, Valim Valéria, Vitali Claudio, Vivino Frederick, Wahren-Herlenius Marie, Moutsopoulos Haralampos M., International Task Force on Nomenclature of Sjögren Disease
Dátum:2025
ISSN:1759-4790 1759-4804
Megjegyzések:Nomenclature for the disease widely known as Sjögren syndrome has proven unsatisfactory. Patients have perceived ♭syndrome' as indicative of a vague collection of symptoms, prompting the Sjögren's Foundation to abandon the term. Furthermore, the traditional distinction between ♭primary' and ♭secondary' forms fails to account for the complex interplay between overlapping autoimmune diseases. Following a bibliometric analysis, systematic literature review and a Delphi consensus process with equal involvement of professional and patient representatives, five recommendations are now issued. First, the term ♭Sjögren disease' should replace ♭Sjögren syndrome'. Second, the acronym ♭SjD' should be used as an abbreviation for ♭Sjögren disease'. Third, the descriptor ♭associated' should be used in lieu of ♭secondary' for Sjögren disease occurring in association with a second systemic autoimmune disease for which classification criteria are fulfilled. Fourth, Sjögren disease is the preferred terminology in common parlance and in clinical diagnosis, without differentiation as to primary and associated forms. Fifth, the differentiation between primary and associated Sjögren is recommended for scientific studies to define a homogeneous population. In conclusion, the consensus endorses ♭Sjögren disease' as the official nomenclature to acknowledge the distinct pathogenesis of this disorder and to improve clarity in both clinical practice and research.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Consensus
Delphi Technique
Humans
Sjogren's Syndrome
Terminology as Topic
Megjelenés:Nature Reviews Rheumatology. - 21 : 7 (2025), p. 426-437. -
További szerzők:Baer, Alan N. Brito-Zerón, Pilar Hammitt, Katherine M. Bouillot, Coralie Retamozo, Soledad Mackey, Alison Yarowsky, David Turner, Breck Blanck, Jaime Fisher, Benjamin Akpek, Esen K. Baldini, Chiara Bootsma, Hendrika Bowman, Simon J. Dörner, Thomas Laing, Leslie Lieberman, Scott M. Mariette, Xavier Pflugfelder, Stephen C. Sankar, Vidya Sisó-Almirall, Antoni Tzioufas, Athanasios G. Anaya, Juan-Manuel Armagan, Berkan Bombardieri, Michele Carsons, Steven E. Vita, Salvatore de Fox, Robert I. Gerli, Roberto Giacomelli, Roberto Gottenberg, Jacques-Eric Hernandez-Molina, Gabriela Jonsson, Roland Kruize, Aike A. Kwok, Seung-Ki Li, Xiaomei McCoy, Sara S. Ng, Wan Fai Olsson, Peter Rischmueller, Maureen Saraux, Alain Scofield, R. Hal Valim, Valeria Vitali, Claudio Vivino, Frederick Wahren-Herlenius, Marie Moutsopoulos, Haralampos M. Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) International Task Force on Nomenclature of Sjögren Disease
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Intézményi repozitóriumban (DEA) tárolt változat
Borító:

12.

001-es BibID:BIBFORM100739
035-os BibID:(WOS)000753131900023 (Scopus)85113866014
Első szerző:Ramos-Casals, Manuel
Cím:Childhood-onset of primary Sjögren's syndrome : phenotypic characterization at diagnosis of 158 children / Ramos-Casals Manuel, Acar-Denizli Nihan, Vissink Arjan, Brito-Zerón Pilar, Li Xiaomei, Carubbi Francesco, Priori Roberta, Toplak Nataŝa, Baldini Chiara, Faugier-Fuentes Enrique, Kruize Aike A., Mandl Thomas, Tomiita Minako, Gandolfo Saviana, Hashimoto Kunio, Hernandez-Molina Gabriela, Hofauer Benedikt, Mendieta-Zerón Samara, Rasmussen Astrid, Sandhya Pulukool, Sene Damien, Trevisani Virginia Fernandes Moca, Isenberg David, Sundberg Erik, Pasoto Sandra G., Sebastian Agata, Suzuki Yasunori, Retamozo Soledad, Xu Bei, Giacomelli Roberto, Gattamelata Angelica, Bizjak Masa, Bombardieri Stefano, Loor-Chavez Richard-Eduardo, Hinrichs Anneline, Olsson Peter, Bootsma Hendrika, Lieberman Scott M., Sjogren Big Data Consortium
Dátum:2021
ISSN:1462-0324 1462-0332
Megjegyzések:Objectives: To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS. Methods: The Big Data Sjögren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 years according to the fulfilment of the 2002/2016 classification criteria. Results: Among the 12 083 patients included in the Sjögren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2 years): 126 (80%) reported dry mouth, 111 (70%) dry eyes, 52 (33%) parotid enlargement, 118/122 (97%) positive minor salivary gland biopsy and 60/64 (94%) abnormal salivary US study, 140/155 (90%) positive ANA, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive RF. The systemic EULAR Sjögren's syndrome disease activity index (ESSDAI) domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SS showed the highest mean ESSDAI score and the highest frequencies of systemic disease in 5 (constitutional, lymphadenopathy, glandular, cutaneous and haematological) of the 12 ESSDAI domains, and the lowest frequencies in 4 (articular, pulmonary, peripheral nerve and CNS) in comparison with patients with adult-onset disease. Conclusions: Childhood-onset primary SS involves around 1% of patients with primary SS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role in modulating the phenotypic expression of the disease.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjogren's syndrome
autoimmune diseases
childhood
epidemiology
paediatrics
Megjelenés:Rheumatology. - 60 : 10 (2021), p. 4558-4567. -
További szerzők:Acar-Denizli, Nihan Vissink, Arjan Brito-Zerón, Pilar Li, Xiaomei Carubbi, Francesco Priori, Roberta Toplak, Nataŝa Baldini, Chiara Faugier-Fuentes, Enrique Kruize, Aike A. Mandl, Thomas Tomiita, Minako Gandolfo, Saviana Hashimoto, Kunio Hernandez-Molina, Gabriela Hofauer, Benedikt Mendieta-Zerón, Samara Rasmussen, Astrid Sandhya, Pulukool Sene, Damien Trevisani, Virginia Fernandes Moça Isenberg, David A. Sundberg, Erik Pasoto, Sandra Sebastian, Agata Suzuki, Yasunori Retamozo, Soledad Xu, Bei Giacomelli, Roberto Gattamelata, Angelica Bizjak, Masa Bombardieri, Stefano Loor-Chavez, Richard-Eduardo Hinrichs, Anneline Olsson, Peter Bootsma, Hendrika Lieberman, Scott M. Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Sjögren Big Data Consortium
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