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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
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2.

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, T. 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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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
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4.

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 Szegedi Gyula (1936-2013) (belgyógyász, immunológus) Szekanecz Zoltán (1964-) (reumatológus, belgyógyász, immunológus)
Internet cím:elektronikus változat
elektronikus változat
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5.

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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6.

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