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dc.contributor.author
Brito Zerón, Pilar
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dc.contributor.author
Acar Denizli, Nihan
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Ng, Wan Fai
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Zeher, Margit
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Rasmussen, Astrid
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Mandl, Thomas
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Seror, Raphaele
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Xiaolin, Li
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Baldini, Chiara
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Gottenberg, Jaques
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Danda, Debashish
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Quartuccio, Luca
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Priori, Roberta
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Hernandez Molina, Gabriela
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Armagan, Berkan
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Kruize, Aike
dc.contributor.author
Kwok, Seung Ki
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Kvarnström, Marika
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Praprotnik, Sonja
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Sene, Damien
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Bartoloni, Elena
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Solans, R.
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Rischmueller, M.
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Suzuki, Y.
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Isenberg, D. A.
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Valim, V.
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Wiland, P.
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Nordmark, G.
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Fraile, G.
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Retamozo, Maria Soledad
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dc.date.available
2021-08-31T01:28:27Z
dc.date.issued
2018-09-14
dc.identifier.citation
Brito Zerón, Pilar; Acar Denizli, Nihan; Ng, Wan Fai; Zeher, Margit; Rasmussen, Astrid; et al.; How immunological profle drives clinical phenotype of primary Sjögren’s syndrome at diagnosis: analysis of 10,500 patients (Sjögren Big Data Project); Clinical & Exper Rheumatology; Clinical And Experimental Rheumatology; 36; 3; 14-9-2018; 112
dc.identifier.issn
0392-856X
dc.identifier.uri
http://hdl.handle.net/11336/139263
dc.description.abstract
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.
dc.format
application/pdf
dc.language.iso
eng
dc.publisher
Clinical & Exper Rheumatology
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dc.rights
info:eu-repo/semantics/openAccess
dc.rights.uri
https://creativecommons.org/licenses/by-nc-sa/2.5/ar/
dc.subject
Primary Sjögren’s syndrome
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Salivary gland biopsy
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Ro/La autoantibodies
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Hypocomplementaemia
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Cryoglobulinaemia
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Reumatología
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dc.subject.classification
Medicina Clínica
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dc.subject.classification
CIENCIAS MÉDICAS Y DE LA SALUD
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dc.title
How immunological profle drives clinical phenotype of primary Sjögren’s syndrome at diagnosis: analysis of 10,500 patients (Sjögren Big Data Project)
dc.type
info:eu-repo/semantics/article
dc.type
info:ar-repo/semantics/artículo
dc.type
info:eu-repo/semantics/publishedVersion
dc.date.updated
2019-10-22T16:40:51Z
dc.identifier.eissn
1593-098X
dc.journal.volume
36
dc.journal.number
3
dc.journal.pagination
112
dc.journal.pais
Italia
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dc.journal.ciudad
Pisa
dc.description.fil
Fil: Brito Zerón, Pilar. Hospital Sanitas CIMA; España. Universidad de Barcelona; España
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Fil: Acar Denizli, Nihan. Mimar Sinan Fine Arts University; Turquía
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Fil: Ng, Wan Fai. University of Newcastle; Reino Unido
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Fil: Zeher, Margit. University of Debrecen; Hungría
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Fil: Rasmussen, Astrid. Oklahoma Medical Research Foundation; Estados Unidos
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Fil: Mandl, Thomas. Lund University; Suecia
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Fil: Seror, Raphaele. Université Paris Sud; Francia
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Fil: Xiaolin, Li. Anhui Provincial Hospital; China
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Fil: Baldini, Chiara. Università degli Studi di Pisa; Italia
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Fil: Gottenberg, Jaques. Université de Strasbourg; Francia. Centre National de la Recherche Scientifique; Francia
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Fil: Danda, Debashish. Christian Medical College & Hospital; India
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Fil: Quartuccio, Luca. University Hospital “Santa María della Misericordia”; Italia
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Fil: Priori, Roberta. Università degli Studi di Roma "La Sapienza"; Italia
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Fil: Hernandez Molina, Gabriela. Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán; México
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Fil: Armagan, Berkan. Hacettepe University. Faculty of Medicine.Department of Internal Medicine; Turquía
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Fil: Kruize, Aike. University Medical Center Utrecht; Países Bajos
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Fil: Kwok, Seung Ki. The Catholic University of Korea; Corea del Sur
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Fil: Kvarnström, Marika. Karolinska University Hospital.Department of Medicine.Unit of Rheumatology. Karolinska Institutet ; Suecia
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Fil: Praprotnik, Sonja. University Medical Centre; Eslovenia
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Fil: Sene, Damien. Université Paris Diderot - Paris 7; Francia
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Fil: Bartoloni, Elena. Università di Perugia; Italia
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Fil: Solans, R.. Hospital Vall d’Hebron; Italia
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Fil: Rischmueller, M.. University of Western Australia; Australia
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Fil: Suzuki, Y.. Kanazawa University Hospital; Japón
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Fil: Isenberg, D. A.. University College London; Estados Unidos
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Fil: Valim, V.. Federal University of Espírito Santo; Brasil
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Fil: Wiland, P.. Wroclaw Medical Hospital; Polonia
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Fil: Nordmark, G.. Uppsala Universitet; Suecia
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Fil: Fraile, G.. Hospital Ramón y Cajal; España
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Fil: Retamozo, Maria Soledad. Consejo Nacional de Investigaciones Científicas y Técnicas. Centro Científico Tecnológico Conicet - Córdoba. Instituto de Investigaciones en Ciencias de la Salud. Universidad Nacional de Córdoba. Instituto de Investigaciones en Ciencias de la Salud; Argentina. Hospital Privado Centro Medico de Córdoba; Argentina; Argentina. Instituto Universitario de Ciencias Biomédicas de Córdoba; Argentina
dc.journal.title
Clinical And Experimental Rheumatology
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dc.relation.alternativeid
info:eu-repo/semantics/altIdentifier/url/https://www.clinexprheumatol.org/abstract.asp?a=12899
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