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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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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
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