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1.
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
Internet cím:
Szerző által megadott URL
Intézményi repozitóriumban (DEA) tárolt változat
Borító:
Saját polcon:
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, Özkzltas 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
Özkzltaș, 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
Internet cím:
Szerző által megadott URL
DOI
Intézményi repozitóriumban (DEA) tárolt változat
Borító:
Saját polcon:
3.
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
Internet cím:
Szerző által megadott URL
DOI
Intézményi repozitóriumban (DEA) tárolt változat
Borító:
Saját polcon:
4.
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
Internet cím:
Szerző által megadott URL
Intézményi repozitóriumban (DEA) tárolt változat
Borító:
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