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1.
001-es BibID:
BIBFORM083560
035-os BibID:
(WOS)000574394600030 (Scopus)85078897832
Első szerző:
Brito-Zerón, Pilar
Cím:
Epidemiological profile and north-south gradient driving baseline systemic involvement of primary Sjögren's syndrome / Pilar Brito-Zerón, Nihan Acar-Denizli, Wan-Fai Ng, Ildiko Fanny Horváth, Astrid Rasmussen, Raphaele Seror, Xiaomei Li, Chiara Baldini, Jacques-Eric Gottenberg, Debashish Danda, Luca Quartuccio, Roberta Priori, Gabriela Hernandez-Molina, Berkan Armagan, Aike A. Kruize, Seung-Ki Kwok, Marika Kvarnstrom, Sonja Praprotnik, Damien Sene, Roberto Gerli, Roser Solans, Maureen Rischmueller, Thomas Mandl, Yasunori Suzuki, David Isenberg, Valeria Valim, Piotr Wiland, Gunnel Nordmark, Guadalupe Fraile, Hendrika Bootsma, Hideki Nakamura, Roberto Giacomelli, Valerie Devauchelle-Pensec, Benedikt Hofauer, Michele Bombardieri, Virginia Fernandes Moça Trevisani, Daniel Hammenfors, Sandra G. Pasoto, Soledad Retamozo, Tamer A. Gheita, Fabiola Atzeni, Jacques Morel, Cristina Vollenweider, Margit Zeher, Kathy Sivils, Bei Xu, Stefano Bombardieri, Pulukool Sandhya, Salvatore De Vita, Antonina Minniti, Jorge Sánchez-Guerrero, Levent Kilic, Eefje van der Heijden, Sung-Hwan Park, Marie Wahren-Herlenius, Xavier Mariette, Manuel Ramos-Casals, Sjögren Big Data Consortium
Dátum:
2020
ISSN:
1462-0324 1462-0332
Megjegyzések:
OBJECTIVE: To characterize the systemic phenotype of primary Sjögren's syndrome at diagnosis by analysing the EULAR-SS disease activity index (ESSDAI) scores. METHODS: The Sjögren Big Data Consortium is an international, multicentre registry based on worldwide data-sharing cooperative merging of pre-existing databases from leading centres in clinical research in Sjögren's syndrome from the five continents. RESULTS: The cohort included 10 007 patients (9352 female, mean 53?years) with recorded ESSDAI scores available. At diagnosis, the mean total ESSDAI score was 6.1; 81.8% of patients had systemic activity (ESSDAI score ?1). Males had a higher mean ESSDAI (8.1 vs 6.0, P?<?0.001) compared with females, as did patients diagnosed at <35?years (6.7 vs 5.6 in patients diagnosed at >65?years, P?<?0.001). The highest global ESSDAI score was reported in Black/African Americans, followed by White, Asian and Hispanic patients (6.7, 6.5, 5.4 and 4.8, respectively; P?<?0.001). The frequency of involvement of each systemic organ also differed between ethnic groups, with Black/African American patients showing the highest frequencies in the lymphadenopathy, articular, peripheral nervous system, CNS and biological domains, White patients in the glandular, cutaneous and muscular domains, Asian patients in the pulmonary, renal and haematological domains and Hispanic patients in the constitutional domain. Systemic activity measured by the ESSDAI, clinical ESSDAI (clinESSDAI) and disease activity states was higher in patients from southern countries (P?<?0.001). CONCLUSION: The systemic phenotype of primary Sjögren's syndrome is strongly influenced by personal determinants such as age, gender, ethnicity and place of residence, which are key geoepidemiological players in driving the expression of systemic disease at diagnosis.
Tárgyszavak:
Orvostudományok
Klinikai orvostudományok
idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
ethnicity
gender
geoepidemiology
phenotype
primary Sjögren's syndrome
Megjelenés:
Rheumatology. - 59 : 9 (2020), p. 2350-2359. -
További szerzők:
Acar-Denizli, Nihan
Ng, Wan Fai
Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus)
Rasmussen, Astrid
Seror, Raphaele
Li, Xiaomei
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
Gerli, Roberto
Solans, Roser
Rischmueller, Maureen
Mandl, Thomas
Suzuki, Yasunori
Isenberg, David A.
Valim, Valeria
Wiland, Piotr
Nordmark, Gunnel
Fraile, Guadalupe
Bootsma, Hendrika
Nakamura, Hideki
Giacomelli, Roberto
Devauchelle-Pensec, Valerie
Hofauer, Benedikt
Bombardieri, Michele
Trevisani, Virginia Fernandes Moça
Hammenfors, Daniel
Pasoto, Sandra
Retamozo, Soledad
Gheita, Tamer A.
Atzeni, Fabiola
Morel, Jacques
Vollenveider, Cristina
Zeher Margit (1957-2018) (belgyógyász, allergológus és klinikai immunológus, reumatológus)
Sivils, Kathy
Xu, Bei
Bombardieri, Stefano
Sandhya, Pulukool
Vita, Salvatore de
Minniti, Antonina
Sanchez-Guerrero, Jorge
Kilic, Levent
Heijden, Eefje van der
Park, Sung-Hwan
Wahren-Herlenius, Marie
Mariette, Xavier
Ramos-Casals, Manuel
Sjögren Big Data Consortium
Internet cím:
Szerző által megadott URL
DOI
Intézményi repozitóriumban (DEA) tárolt változat
Borító:
Saját polcon:
2.
001-es BibID:
BIBFORM100739
035-os BibID:
(WOS)000753131900023 (Scopus)85113866014
Első szerző:
Ramos-Casals, Manuel
Cím:
Childhood-onset of primary Sjögren's syndrome : phenotypic characterization at diagnosis of 158 children / Ramos-Casals Manuel, Acar-Denizli Nihan, Vissink Arjan, Brito-Zerón Pilar, Li Xiaomei, Carubbi Francesco, Priori Roberta, Toplak Nataŝa, Baldini Chiara, Faugier-Fuentes Enrique, Kruize Aike A., Mandl Thomas, Tomiita Minako, Gandolfo Saviana, Hashimoto Kunio, Hernandez-Molina Gabriela, Hofauer Benedikt, Mendieta-Zerón Samara, Rasmussen Astrid, Sandhya Pulukool, Sene Damien, Trevisani Virginia Fernandes Moca, Isenberg David, Sundberg Erik, Pasoto Sandra G., Sebastian Agata, Suzuki Yasunori, Retamozo Soledad, Xu Bei, Giacomelli Roberto, Gattamelata Angelica, Bizjak Masa, Bombardieri Stefano, Loor-Chavez Richard-Eduardo, Hinrichs Anneline, Olsson Peter, Bootsma Hendrika, Lieberman Scott M., Sjogren Big Data Consortium
Dátum:
2021
ISSN:
1462-0324 1462-0332
Megjegyzések:
Objectives: To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS. Methods: The Big Data Sjögren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19 years according to the fulfilment of the 2002/2016 classification criteria. Results: Among the 12 083 patients included in the Sjögren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2 years): 126 (80%) reported dry mouth, 111 (70%) dry eyes, 52 (33%) parotid enlargement, 118/122 (97%) positive minor salivary gland biopsy and 60/64 (94%) abnormal salivary US study, 140/155 (90%) positive ANA, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive RF. The systemic EULAR Sjögren's syndrome disease activity index (ESSDAI) domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SS showed the highest mean ESSDAI score and the highest frequencies of systemic disease in 5 (constitutional, lymphadenopathy, glandular, cutaneous and haematological) of the 12 ESSDAI domains, and the lowest frequencies in 4 (articular, pulmonary, peripheral nerve and CNS) in comparison with patients with adult-onset disease. Conclusions: Childhood-onset primary SS involves around 1% of patients with primary SS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role in modulating the phenotypic expression of the disease.
Tárgyszavak:
Orvostudományok
Klinikai orvostudományok
idegen nyelvű folyóiratközlemény külföldi lapban
folyóiratcikk
Sjogren's syndrome
autoimmune diseases
childhood
epidemiology
paediatrics
Megjelenés:
Rheumatology. - 60 : 10 (2021), p. 4558-4567. -
További szerzők:
Acar-Denizli, Nihan
Vissink, Arjan
Brito-Zerón, Pilar
Li, Xiaomei
Carubbi, Francesco
Priori, Roberta
Toplak, Nataŝa
Baldini, Chiara
Faugier-Fuentes, Enrique
Kruize, Aike A.
Mandl, Thomas
Tomiita, Minako
Gandolfo, Saviana
Hashimoto, Kunio
Hernandez-Molina, Gabriela
Hofauer, Benedikt
Mendieta-Zerón, Samara
Rasmussen, Astrid
Sandhya, Pulukool
Sene, Damien
Trevisani, Virginia Fernandes Moça
Isenberg, David A.
Sundberg, Erik
Pasoto, Sandra
Sebastian, Agata
Suzuki, Yasunori
Retamozo, Soledad
Xu, Bei
Giacomelli, Roberto
Gattamelata, Angelica
Bizjak, Masa
Bombardieri, Stefano
Loor-Chavez, Richard-Eduardo
Hinrichs, Anneline
Olsson, Peter
Bootsma, Hendrika
Lieberman, Scott M.
Horváth Ildikó Fanny (1980-) (belgyógyász, allergológus, klinikai immunológus)
Szántó Antónia (1977-) (belgyógyász, allergológus és klinikai immunológus)
Sjögren Big Data Consortium
Internet cím:
Szerző által megadott URL
DOI
Intézményi repozitóriumban (DEA) tárolt változat
Borító:
Saját polcon:
3.
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:
4.
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
Internet cím:
Szerző által megadott URL
Intézményi repozitóriumban (DEA) tárolt változat
Borító:
Saját polcon:
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