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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:
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