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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:BIBFORM103399
035-os BibID:(Scopus)85149171419 (WoS)000952863200010
Első szerző:Béldi Tibor (orvos)
Cím:The effect of COVID-19 pandemic on idiopathic inflammatory myositis patients : a single centre experience / Béldi Tibor, Vincze Anett, Miltényi-Szabó Balázs, Varga Zsófia, Szabó Katalin, Griger Zoltán, Nagy-Vincze Melinda
Dátum:2023
ISSN:0392-856X 1593-098X
Megjegyzések:Objectives: Pandemic caused by coronavirus disease (COVID-19) determines the life of clinicians and patients since 2 years. We have a lot of information about disease course, treatment and protection against virus, but less on the prognosis of infection in patients with idiopathic inflammatory myopathies (IIM). Also few data are available on triggered humoral response and side effects after vaccination. Methods: Our goal was to assess by a retrospective cross-sectional study the above data in our cohort (176 IIM patients) by identifying COVID-19 positive patients and follow disease course. Incidence and complications of vaccination were determined by questionnaires. 101 patients volunteered for complex blood test. Results: By June 1st, 2021 significantly higher incidence of COVID 19 infections (34.7%) were identified comparing to the national prevalence (8.2%). A third of these infections occurred asymptomatically or mild. Patients requiring hospitalisation had a significantly longer disease duration and a higher incidence of anti-Jo-1 antibody. All patients infected by COVID-19 became seropositive regardless the immunosuppressive therapy or symptoms severity. 54.3% of the patients received anti-COVID-19 vaccine. 72.3% of patients became seropositive after vaccination. Higher antibody titer against spike protein was detected after Pfizer-BioNTech vaccination compared to others. Patients receiving steroid therapy had decreased post-vaccination antibody response compared to those without steroid treatment. No major post-vaccination infection was observed. Conclusions: Based on our results, myositis may be associated with an increased risk of COVID-19 infection. Independent risk factor for hospitalisation are longer disease duration and anti-Jo1 positivity. Anti-SARS-CoV2 vaccines seem safe and tolerable and strongly recommended for that population.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
myositis
interstitial lung disease
COVID infection
vaccination
Megjelenés:Clinical And Experimental Rheumatology. - 41 : 2 (2023), p. 254-260. -
További szerzők:Vincze Anett (1993-) (orvos) Miltényi-Szabó Balázs (1996-) (orvostanhallgató) Varga Zsófia (1992-) (molekuláris biológus) Szabó Katalin (1991-) (orvos) Griger Zoltán (1979-) (belgyógyász, allergológus és klinikai immunológus, reumatológus) Nagy-Vincze Melinda (1985-) (orvos)
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3.

001-es BibID:BIBFORM128093
035-os BibID:(scopus)86000149599
Első szerző:Bishnoi, Anuradha
Cím:The Myositis Clinical Trials Consortium : an international collaborative initiative to promote clinical trials in adult and juvenile myositis / Bishnoi Anuradha, Tang Iris Yan Ki, Yoshida Akira, Pais Faye M., Usman Sabeena Y., Dominguez Solciris A., Kavadichanda Chengappa G., Rivero-Gallegos Daphne, Dourado Eduardo, Conticini Edoardo, Bozán Francisca, Tulluru Gayathri, Lilleker James B., Sreerama Reddy Kaushik, Landon-Cardinal Océane, Smaili Rachid, Keret Shiri, Khoo Thomas, Lan Ting-Yuan, Leclair Valérie, Oddis Chester V., Vencovsky Jirí, Kuwana Masataka, Gandiga Prateek C., Aggarwal Rohit, Myositis Clinical Trials Consortium investigators
Dátum:2025
ISSN:0392-856X 1593-098X
Megjegyzések:Idiopathic inflammatory myopathies (IIM), or myositis, are a heterogeneous group of systemic autoimmune disorders that are associated with significant morbidity and mortality. Conducting high-quality clinical trials in IIM is challenging due to the rare and variable presentations of disease. To address this challenge, the Myositis Clinical Trials Consortium (MCTC) was formed. MCTC is a collaborative international alliance dedicated to facilitating, promoting, coordinating and conducting clinical trials and related research in IIM. This partnership works to advance the discovery of effective evidence-based treatments for IIM by integrating a diverse group of clinical investigators, research professionals, medical centres, patient groups, and industry partners. The Steering Committee, Core Group, and Paediatric Subcommittee of MCTC are comprised of myositis experts and junior investigators from around the world, representing a diversity of genders, geographies, and subspecialties. MCTC works alongside other current myositis organisations to complement existing work by concentrating on the operationalisation of clinical trials. Our pilot Myositis Investigators' Information Survey gathered responses from 173 myositis investigators globally and found considerable variability in proficiency with outcome measures, geographic disparities in patient recruitment, and a significant disconnect between investigators' routine myositis patient load and clinical trial enrolment. MCTC will meet the need to support and diversify myositis clinical trials by facilitating trial planning, feasibility assessments, site selection, and the training and mentoring of junior investigators/centres to establish their readiness for clinical trial participation. Through experienced leadership, strategic collaborations, and interdisciplinary discussions, MCTC will establish standards for IIM clinical trial design, protocols, and outcome measures in myositis.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
myositis
rare diseases
clinical trials
outcome assessment
organisation and administration
Megjelenés:Clinical And Experimental Rheumatology. - 43 : 2 (2025), p. 202-210. -
További szerzők:Tang, Iris Yan Ki Yoshida, Akira Pais, Faye M. Usman, Sabeena Y. Dominguez, Solciris A. Kavadichanda, Chengappa G. Rivero-Gallegos, Daphne Dourado, Eduardo Conticini, Edoardo Bozán, Francisca Tulluru, Gayathri Lilleker, James B. Sreerama Reddy, Kaushik Landon-Cardinal, Océane Smaili, Rachid Keret, Shiri Khoo, Thomas Lan, Ting-Yuan Leclair, Valérie Oddis, Chester V. Vencovsky, Jiri Kuwana, Masataka Gandiga, Prateek C. Aggarwal, Rohit Griger Zoltán (1979-) (belgyógyász, allergológus és klinikai immunológus, reumatológus) Myositis Clinical Trials Consortium investigators
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4.

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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5.

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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6.

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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7.

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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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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9.

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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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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Borító:

11.

001-es BibID:BIBFORM006925
Első szerző:Lakos Gabriella (laboratóriumi szakorvos, transzfúziológus, immunológus)
Cím:Anti-cyclic citrullinated peptide antibody isotypes in rheumatoid arthritis : association with disease duration, rheumatoid factor production and the presence of shared epitope / Lakos Gabriella, Soós Lilla, Fekete Andrea, Szabó Zoltán, Zeher Margit, Horváth Ildikó Fanny, Dankó Katalin, Kapitány Anikó, Gyetvai Ágnes, Szegedi Gyula, Szekanecz Zoltán
Dátum:2008
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
Megjelenés:Clinical and Experimental Rheumatology. - 26 : 2 (2008), p. 253-260. -
További szerzők:Soós Lilla Fekete Andrea (immunológus) Szabó Zoltán (1970-) (belgyógyász, reumatológus) Zeher Margit (1957-2018) (belgyógyász, allergológus és klinikai immunológus, reumatológus) Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Dankó Katalin (1952-2021) (belgyógyász, allergológus és klinikai immunológus) Kapitány Anikó (1979-) (molekuláris biológus) Gyetvai Ágnes (1978-) (biotechnológus) Szegedi Gyula (1936-2013) (belgyógyász, immunológus) Szekanecz Zoltán (1964-) (reumatológus, belgyógyász, immunológus)
Internet cím:elektronikus változat
elektronikus változat
Borító:

12.

001-es BibID:BIBFORM100734
035-os BibID:(WOS)000731864300024 (Scopus)85122843096
Első szerző:Retamozo, Soledad
Cím:Influence of the age at diagnosis in the disease expression of primary Sjögren syndrome. Analysis of 12,753 patients from the Sjögren Big Data Consortium / S. Retamozo, N. Acar-Denizli, I. F. Horváth, W. F. Ng, A. Rasmussen, X. Dong, X. Li, C. Baldini, P. Olsson, R. Priori, R. Seror, Jacques-Eric Gottenberg, A. A. Kruize, G. Hernandez-Molina, A. Vissink, P. Sandhya, B. Armagan, L. Quartuccio, A. Sebastian, S. Praprotnik, E. Bartoloni, Seung-Ki Kwok, M. Kvarnstrom, M. Rischmueller, R. Soláns-Laqué, D. Sene, S. G. Pasoto, Y. Suzuki, D. A. Isenberg, V. Valim, G. Nordmark, H. Nakamura, V. Fernandes Moca Trevisani, B. Hofauer, A. Sisó-Almirall, R. Giacomelli, V. Devauchelle-Pensec, M. Bombardieri, F. Atzeni, D. Hammenfors, B. Maure, S. E. Carsons, T. Gheita, I. Sánchez-Berná, M. López-Dupla, J. Morel, N. Inanc, E. Fonseca-Aizpuru, C. Morcillo, C. Vollenweider, S. Melchor, M. Vázquez, E. Díaz-Cuiza, S. Consani-Fernández, B. De-Miguel-Campo, A. Szántó, S. Bombardieri, A. Gattamelata, A. Hinrichs, J. Sánchez-Guerrero, D. Danda, L. Kilic, S. De Vita, P. Wiland, R. Gerli, S. H. Park, M. Wahren-Herlenius, H. Bootsma, X. Mariette, M. Ramos-Casals, P. Brito-Zerón
Dátum:2021
ISSN:0392-856X
Megjegyzések:Objectives: To analyse how the main components of the disease phenotype (sicca symptoms, diagnostic tests, immunological markers and systemic disease) can be driven by the age at diagnosis of primary Sjögren's syndrome (pSS). Methods: By January 2021, the participant centres had included 12,753 patients from 25 countries that fulfilled the 2002/2016 classification criteria for pSS. The age at diagnosis was defined as the time when the attending physician confirmed fulfilment of the criteria. Patients were clustered according to age at diagnosis. 50 clusters with more than 100 observations (from 27 to 76 years) were used to study the influence of the age at diagnosis in the disease expression. Results: There was a consistent increase in the frequency of oral dryness according to the age at diagnosis, with a frequency of <90% in patients diagnosed at the youngest ages and >95% in those diagnosed at the oldest ages. The smooth curves that best fitted a linear model were the frequency of dry mouth (adjusted R2 0.87) and the frequency of abnormal oral tests (adjusted R2 0.72). Therefore, for each 1-year increase in the age at diagnosis, the frequency of dry mouth increased by 0.13%, and the frequency of abnormal oral diagnostic tests by 0.11%. There was a consistent year-by-year decrease in the frequency of all autoantibodies and immunological markers except for cryoglobulins. According to the linear models, for each 1-year increase in the age at diagnosis, the frequency of a positive result decreased by 0.57% (for anti-Ro antibodies), 0.47% (for RF) and 0.42% (for anti-La antibodies). The ESSDAI domains which showed a more consistent decrease were glandular and lymph node involvement (for each 1-year increase in the age at diagnosis, the frequency of activity decreased by 0.18%), and constitutional, cutaneous, and haematological involvements (the frequency decreased by 0.09% for each 1-year increase). In contrast, other domains showed an ascending pattern, especially pulmonary involvement (for each 1-year increase in the age at diagnosis, the frequency of activity increased by 0.22%), and peripheral nerve involvement (the frequency increased by 0.09% for each 1-year increase). Conclusions: The influence of the age at diagnosis on the key phenotypic features of pSS is strong, and should be considered critical not only for designing a personalised diagnostic approach, but also to be carefully considered when analysing the results of diagnostic tests and immunological parameters, and when internal organ involvement is suspected at diagnosis.
Tárgyszavak:Orvostudományok Klinikai orvostudományok idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's syndrome
age
disease phenotype
immunological markers
Megjelenés:Clinical and Experimental Rheumatology. - 39 : 6 (2021), p. 166-174. -
További szerzők:Acar-Denizli, Nihan Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus) Ng, Wan Fai Rasmussen, Astrid Dong, X. Li, X. Baldini, Chiara Olsson, Peter Priori, Roberta Seror, Raphaele Gottenberg, Jacques-Eric Kruize, Aike A. Hernandez-Molina, Gabriela Vissink, Arjan Sandhya, Pulukool Armagan, Berkan Quartuccio, Luca Sebastian, Agata Praprotnik, Sonja Bartoloni, Elena Kwok, Seung-Ki Kvarnstrom, Marika Rischmueller, Maureen Soláns-Laqué, Roser Sene, Damien Pasoto, Sandra Suzuki, Yasunori Isenberg, David A. Valim, Valeria Nordmark, Gunnel Nakamura, Hideki Fernandes Moça Trevisani, Virginia Hofauer, Benedikt Sisó-Almirall, Antoni Giacomelli, Roberto Devauchelle-Pensec, Valerie Bombardieri, Michele Atzeni, F. Hammenfors, Daniel Maure, B. Carsons, Steven E. Gheita, Tamer A. Sánchez-Berná, I. López-Dupla, Miguel Morel, Jacques Inanc, Nevsun Fonseca-Aizpuru, Eva Morcillo, C. Vollenveider, Cristina Melchor, Sheila Vázquez, Marta Diaz-Cuiza, E. Consani-Fernández, S. de-Miguel-Campo, B. Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus) Bombardieri, Stefano Gattamelata, Angelica Hinrichs, Anneline Sanchez-Guerrero, Jorge Danda, Debashish Kilic, Levent Vita, Salvatore de Wiland, Piotr Gerli, Roberto Park, S. H. Wahren-Herlenius, Marie Bootsma, Hendrika Mariette, Xavier Ramos-Casals, Manuel Brito-Zerón, Pilar
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