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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:
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
Internet cím:
Szerző által megadott URL
DOI
Intézményi repozitóriumban (DEA) tárolt változat
Borító:
Saját polcon:
3.
001-es BibID:
BIBFORM104986
035-os BibID:
(WOS)000786680100001 (Scopus)85139097101
Első szerző:
Hernandez-Molina, Gabriela
Cím:
Characterization and outcomes of 414 patients with primary SS who developed haematological malignancies / Hernández-Molina Gabriela, Kostov Belchin, Brito-Zerón Pilar, Vissink Arjan, Mandl Thomas, Hinrichs Anneline C., Quartuccio Luca, Baldini Chiara, Seror Raphaele, Szántó Antonia, Isenberg David, Gerli Roberto, Nordmark Gunnel, Rasmussen Astrid, Solans-Laque Roser, Hofauer Benedikt, Sene Damien, Pasoto Sandra G., Rischmueller Maureen, Praprotnik Sonja, Gheita Tamer A., Danda Debashish, Armagan Berkan, Suzuki Yasunori, Valim Valeria, Devauchelle-Pensec Valerie, Retamozo Soledad, Kvarnstrom Marika, Sebastian Agata, Atzeni Fabiola, Giacomelli Roberto, Carsons Steven E., Kwok Seung-Ki, Nakamura Hideki, Fernandes Moca Trevisani Virginia, Flores-Chávez Alejandra, Mariette Xavier, Ramos-Casals Manuel, Sjögren Big Data Consortium
Dátum:
2023
ISSN:
1462-0324 1462-0332
Megjegyzések:
Abstract Objective To characterize 414 patients with primary SS who developed haematological malignancies and to analyse how the main SS- and lymphoma-related features can modify the presentation patterns and outcomes. Methods By January 2021, the Big Data Sjögren Project Consortium database included 11?966 patients fulfilling the 2002/2016 classification criteria. Haematological malignancies diagnosed according to the World Health Organization (WHO) classification were retrospectively identified. Results There were 414 patients (355 women, mean age 57?years) with haematological malignancies (in 43, malignancy preceded at least one year the SS diagnosis). A total of 376 (91%) patients had mature B-cell malignancy, nearly half had extranodal marginal zone lymphoma (MZL) of mucosa-associated lymphoid tissue (MALT lymphoma) (n?=?197), followed by diffuse large B-cell lymphoma (DLBCL) (n?=?67), nodal MZL lymphoma (n?=?29), chronic lymphocytic leukemia/small lymphocytic lymphoma (CLL/SLL) (n?=?19) and follicular lymphoma (FL) (n?=?17). Rates of complete response, relapses and death were 80%, 34% and 13%, respectively, with a 5-year survival rate of 86.5% after a mean follow-up of 8?years. There were significant differences in age at diagnosis (younger in MALT, older in CLL/SLL), predominant clinical presentation (glandular enlargement in MALT lymphoma, peripheral lymphadenopathy in nodal MZL and FL, constitutional symptoms in DLBCL, incidental diagnosis in CLL/SLL), therapeutic response (higher in MALT lymphoma, lower in DLBCL) and survival (better in MALT, nodal MZL and FL, worse in DLBCL). Conclusion In the largest reported study of haematological malignancies complicating primary SS, we confirm the overwhelming predominance of B-cell lymphomas, especially MALT, with the salivary glands being the primary site of involvement. This highly-specific histopathological scenario is linked with the overall good prognosis with a 5-year survival rate of nearly 90%.
Tárgyszavak:
Orvostudományok
Klinikai orvostudományok
idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjögren's Syndrome
haematological malignancy
lymphoproliferative disease
lymphoma
MALT
Megjelenés:
Rheumatology. - 62 : 1 (2023), p. 243-255. -
További szerzők:
Kostov, Belchin
Brito-Zerón, Pilar
Vissink, Arjan
Mandl, Thomas
Hinrichs, Anneline
Quartuccio, Luca
Baldini, Chiara
Seror, Raphaele
Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus)
Isenberg, David A.
Gerli, Roberto
Nordmark, Gunnel
Rasmussen, Astrid
Soláns-Laqué, Roser
Hofauer, Benedikt
Sene, Damien
Pasoto, Sandra
Rischmueller, Maureen
Praprotnik, Sonja
Gheita, Tamer A.
Danda, Debashish
Armagan, Berkan
Suzuki, Yasunori
Valim, Valeria
Devauchelle-Pensec, Valerie
Retamozo, Soledad
Kvarnstrom, Marika
Sebastian, Agata
Atzeni, Fabiola
Giacomelli, Roberto
Carsons, Steven E.
Kwok, Seung-Ki
Nakamura, Hideki
Fernandes Moça Trevisani, Virginia
Flores-Chávez, Alejandra
Mariette, Xavier
Ramos-Casals, Manuel
Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus)
Tarr Tünde (1976-) (belgyógyász, allergológus és klinikai immunológus)
Sjögren Big Data Consortium
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:
Saját polcon:
5.
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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