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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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2.

001-es BibID:BIBFORM115775
035-os BibID:(cikkazonosító)102062 (scopus)85164311201 (wos)001040655800001
Első szerző:Brito-Zerón, Pilar
Cím:Mortality risk factors in primary Sjögren syndrome : a real-world, retrospective, cohort study / Brito-Zerón Pilar, Flores-Chávez Alejandra, Horváth Ildiko Fanny, Rasmussen Astrid, Li Xiaomei, Olsson Peter, Vissink Arjan, Priori Roberta, Armagan Berkan, Hernandez-Molina Gabriela, Praprotnik Sonja, Quartuccio Luca, Inanc Nevsun, Özkiziltas Burcugül, Bartoloni Elena, Sebastian Agata, Romao Vasco C., Solans Roser, Pasoto Sandra G., Rischmueller Maureen, Galisteo Carlos, Suzuki Yasunori, Trevisani Virginia Fernandes Moca, Fugmann Cecilia, González-García Andrés, Carubbi Francesco, Jurcut Ciprian, Shimizu Toshimasa, Retamozo Soledad, Atzeni Fabiola, Hofauer Benedikt, Melchor-Díaz Sheila, Gheita Tamer, López-Dupla Miguel, Fonseca-Aizpuru Eva, Giacomelli Roberto, Vázquez Marcos, Consani Sandra, Akasbi Miriam, Nakamura Hideki, Szántó Antónia, Farris A. Darise, Wang Li, Mandl Thomas, Gattamelata Angelica, Kilic Levent, Pirkmajer Katja Perdan, Abacar Kerem, Tufan Abdurrahman, de Vita Salvatore, Bootsma Hendrika, Ramos-Casals Manuel, Sjögren Big Data Consortium
Dátum:2023
ISSN:2589-5370
Megjegyzések:Background: What baseline predictors would be involved in mortality in people with primary Sjögren syndrome (SjS) remains uncertain. This study aimed to investigate the baseline characteristics collected at the time of diagnosis of SjS associated with mortality and to identify mortality risk factors for all-cause death and deaths related to systemic SjS activity measured by the ESSDAI score. Methods: In this international, real-world, retrospective, cohort study, we retrospectively collected data from 27 countries on mortality and causes of death from the Big Data Sjögren Registry. Inclusion criteria consisted of fulfilling 2002/2016 SjS classification criteria, and exclusion criteria included chronic HCV/HIV infections and associated systemic autoimmune diseases. A statistical approach based on a directed acyclic graph was used, with all-cause and Sjögren-related mortality as primary endpoints. The key determinants that defined the disease phenotype at diagnosis (glandular, systemic, and immunological) were analysed as independent variables. Findings: Between January 1st, 2014 and December 31, 2023, data from 11,372 patients with primary SjS (93.5% women, 78.4% classified as White, mean age at diagnosis of 51.1 years) included in the Registry were analysed. 876 (7.7%) deaths were recorded after a mean follow-up of 8.6 years (SD 7.12). Univariate analysis of prognostic factors for all-cause death identified eight Sjögren-related variables (ocular and oral tests, salivary biopsy, ESSDAI, ANA, anti-Ro, anti-La, and cryoglobulins). The multivariate CPH model adjusted for these variables and the epidemiological features showed that DAS-ESSDAI (high vs no high: HR = 1.68; 95% CI, 1.27-2.22) and cryoglobulins (positive vs negative: HR = 1.72; 95% CI, 1.22-2.42) were independent predictors of all-cause death. Of the 640 deaths with available information detailing the specific cause of death, 14% were due to systemic SjS. Univariate analysis of prognostic factors for Sjögren-cause death identified five Sjögren-related variables (oral tests, clinESSDAI, DAS-ESSDAI, ANA, and cryoglobulins). The multivariate competing risks CPH model adjusted for these variables and the epidemiological features showed that oral tests (abnormal vs normal results: HR = 1.38; 95% CI, 1.01-1.87), DAS-ESSDAI (high vs no high: HR = 1.55; 95% CI, 1.22-1.96) and cryoglobulins (positive vs negative: HR = 1.52; 95% CI, 1.16-2) were independent predictors of SjS-related death. Interpretation: The key mortality risk factors at the time of SjS diagnosis were positive cryoglobulins and a high systemic activity scored using the ESSDAI, conferring a 2-times increased risk of all-cause and SjS-related death. ESSDAI measurement and cryoglobulin testing should be considered mandatory when an individual is diagnosed with SjS.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Cardiovascular
Infection
Lymphoma
Mortality
Sjögren syndrome
Systemic disease
Megjelenés:eClinicalMedicine. - 61 (2023), p. 1-16. -
További szerzők:Flores-Chávez, Alejandra Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Rasmussen, Astrid Li, Xiaomei Olsson, Peter Vissink, Arjan Priori, Roberta Armagan, Berkan Hernandez-Molina, Gabriela Praprotnik, Sonja Quartuccio, Luca Inanc, Nevsun Özkiziltaș, Burcugül Bartoloni, Elena Sebastian, Agata Romão, Vasco C. Solans, Roser Pasoto, Sandra Rischmueller, Maureen Galisteo, Carlos Suzuki, Yasunori Trevisani, Virginia Fernandes Moça Fugmann, Cecilia González-García, Andrés Carubbi, Francesco Jurcut, Ciprian Shimizu, Toshimasa Retamozo, Soledad Atzeni, Fabiola Hofauer, Benedikt Melchor Díaz, S. Gheita, Tamer A. López-Dupla, Miguel Fonseca-Aizpuru, Eva Giacomelli, Roberto Vázquez, Marcos Consani, Sandra Akasbi, Miriam Nakamura, Hideki Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Farris, Darise Wang, Li Mandl, Thomas Gattamelata, Angelica Kilic, Levent Pirkmajer, Katja Perdan Abacar, Kerem Tufan, Abdurrahman Vita, Salvatore de Bootsma, Hendrika Ramos-Casals, Manuel Sjögren Big Data Consortium
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3.

001-es BibID: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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4.

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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5.

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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6.

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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