How immunological profile drives clinical phenotype of primary Sjögren's syndrome at diagnosis: analysis of 10,500 patients (Sjögren Big Data Project)

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How immunological profile drives clinical phenotype of primary Sjögren's syndrome at diagnosis : analysis of 10,500 patients (Sjögren Big Data Project)

Brito-Zeron, P. (författare): Hosp CIMA Sanitas, Dept Med, Autoimmune Dis Unit, Barcelona, Spain;Univ Barcelona, Sjogrens Syndrome Res Grp AGAUR, Lab Autoimmune Dis Josep Font,Hosp Clin, IDIBAPS,CELLEX,Dept Autoimmune Dis,ICMiD, Barcelona, Spain,Hospital CIMA

Acar-Denizli, N. (författare): Mimar Sinan Fine Arts Univ, Fac Sci & Letters, Dept Stat, Istanbul, Turkey,Mimar Sinan Fine Arts University

Ng, W. F. (författare): Newcastle Univ, Inst Cellular Med, Newcastle Upon Tyne, Tyne & Wear, England,University of Newcastle upon Tyne

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Zeher, M. (författare): Univ Debrecen, Fac Med, Div Clin Immunol, Debrecen, Hungary,University of Debrecen

Rasmussen, A. (författare): Oklahoma Med Res Fdn, Arthrit & Clin Immunol Res Program, 825 NE 13th St, Oklahoma City, OK 73104 USA,Oklahoma Medical Research Foundation

Mandl, Thomas (författare): Lund University,Lunds universitet,Internmedicin - epidemiologi,Forskargrupper vid Lunds universitet,Internal Medicine - Epidemiology,Lund University Research Groups,Skåne University Hospital,Lund Univ, Skane Univ Hosp Malmo, Dept Rheumatol, Malmo, Sweden

Seror, R. (författare): Univ Paris Sud, Hop Univ Paris Sud, AP HP, Ctr Immunol Viral Infect & Autoimmune Dis,INSERM, Paris, France,University of Paris-Saclay,Univ Nacl Cordoba, Consejo Nacl Invest Cient & Tecn CONICET, Inst Invest Ciencias Salud INICSA, Hosp Privado Univ Cordoba,IUCBC, Cordoba, Argentina

Li, X. (författare): Anhui Prov Hosp, Dept Rheumatol & Immunol, Hefei, Anhui, Peoples R China,Anhui Provincial Hospital

Baldini, C. (författare): Univ Pisa, Rheumatol Unit, Pisa, Italy,University of Pisa

Gottenberg, J. -E (författare): University of Strasbourg

Danda, D. (författare): Christian Med Coll & Hosp, Dept Clin Immunol & Rheumatol, Vellore, Tamil Nadu, India,Christian Medical College & Hospital,Paris Diderot Univ, Lariboisiere Hosp, AP HP, Dept Internal Med, Paris, France,UCL, Div Med, Ctr Rheumatol, London, England,Univ Bergen, Dept Clin Sci, Bergen, Norway;Haukeland Hosp, Dept Rheumatol, Bergen, Norway

Quartuccio, L. (författare): Univ Hosp Santa Maria della Misericordia, Dept Med Area DAME, Clin Rheumatol, Udine, Italy,University Hospital 'Santa Maria della Misericordia'

Priori, R. (författare): Sapienza Univ Rome, Rheumatol Clin, Dept Internal Med & Med Specialties, Rome, Italy,Sapienza University of Rome

Hernandez-Molina, G. (författare): Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Dept Rheumatol & Immunol, Mexico City, DF, Mexico,Salvador Zubirán National Institute of Health Sciences and Nutrition

Armagan, B. (författare): Hacettepe Univ, Fac Med, Dept Internal Med, Ankara, Turkey,Hacettepe University

Kruize, A. A. (författare): Univ Med Ctr Utrecht, Dept Rheumatol & Clin Immunol, Utrecht, Netherlands,University Medical Center Utrecht

Kwok, S. -K (författare): Catholic University of Korea,Oklahoma Med Res Fdn, Arthrit & Clin Immunol Res Program, 825 NE 13th St, Oklahoma City, OK 73104 USA

Kvarnstrom, M. (författare): Karolinska Institute,Karolinska Institutet,Karolinska University Hospital,Karolinska Inst, Dept Med, Rheumatol Unit, Stockholm, Sweden;Karolinska Univ Hosp, Stockholm, Sweden

Praprotnik, S. (författare): Univ Med Ctr, Dept Rheumatol, Ljubljana, Slovenia,University Medical Centre Ljubljana

Sene, D. (författare): Paris Diderot Univ, Lariboisiere Hosp, AP HP, Dept Internal Med, Paris, France,Paris Diderot University,Univ Hosp Santa Maria della Misericordia, Dept Med Area DAME, Clin Rheumatol, Udine, Italy

Bartoloni, E. (författare): Univ Perugia, Dept Med, Rheumatol Unit, Perugia, Italy,University of Perugia

Solans, R. (författare): Hosp Valle De Hebron, Dept Internal Med, Barcelona, Spain,Vall d'Hebron University Hospital

Rischmueller, M. (författare): Univ Western Australia, Sch Med, Dept Rheumatol, Crawley, Australia,University of Western Australia, Crawley

Suzuki, Y. (författare): Kanazawa Univ Hosp, Div Rheumatol, Kanazawa, Ishikawa, Japan,Kanazawa University Hospital

Isenberg, D. A. (författare): UCL, Div Med, Ctr Rheumatol, London, England,University College London

Valim, V. (författare): Univ Espirito Santo, Dept Med, Vitoria, Brazil;Univ Hosp HUCAM EBSERH, Vitoria, Brazil,Federal University of Espírito Santo,Brest Univ Hosp, Dept Rheumatol, Brest, France,Univ Fed Sao Paulo, Sao Paulo, Brazil

Wiland, P. (författare): Wroclaw Med Hosp, Dept Rheumatol & Internal Med, Wroclaw, Poland,Wroclaw Medical Hospital

Nordmark, Gunnel (författare): Uppsala University,Uppsala universitet,Reumatologi

Fraile, G. (författare): Hosp Ramon & Cajal, Dept Internal Med, Madrid, Spain,Hospital Ramón Y Cajal

Bootsma, H. (författare): Univ Groningen, Univ Med Ctr Groningen, Dept Rheumatol & Clin Immunol, Groningen, Netherlands,University of Groningen

Nakamura, T. (författare): Nagasaki Univ, Grad Sch Biomed Sci, Dept Radiol & Canc Biol, Nagasaki, Japan,Nagasaki University

Giacomelli, R. (författare): Univ Aquila, Sch Med, Clin Unit Rheumatol, Laquila, Italy,University of L'Aquila

Devauchelle-Pensec, V. (författare): Brest Univ Hosp, Dept Rheumatol, Brest, France,University Hospital of Brest

Knopf, A. (författare): Tech Univ Munich, Klinikum Rechts Isar, Otorhinolaryngol Head & Neck Surg, Munich, Germany,Technical University of Munich

Bombardieri, M. (författare): Queen Mary Univ London, Ctr Expt Med & Rheumatol, London, England,Queen Mary University

Trevisani, V. -F (författare): Univ Fed Sao Paulo, Sao Paulo, Brazil,Federal University of São Paulo

Hammenfors, D. (författare): Univ Bergen, Dept Clin Sci, Bergen, Norway;Haukeland Hosp, Dept Rheumatol, Bergen, Norway,Haukeland University Hospital,University of Bergen

Pasoto, S. G. (författare): Univ Sao Paulo, Fac Med, Hosp Clin, Div Rheumatol, Sao Paulo, Brazil,University of São Paulo

Retamozo, S. (författare): Univ Nacl Cordoba, Consejo Nacl Invest Cient & Tecn CONICET, Inst Invest Ciencias Salud INICSA, Hosp Privado Univ Cordoba,IUCBC, Cordoba, Argentina,National University of Cordoba, Argentina

Gheita, T. A. (författare): Cairo Univ, Kasr Al Ainy Sch Med, Dept Rheumatol, Cairo, Egypt,Cairo University

Atzeni, F. (författare): IRCCS Galeazzi Orthopaed Inst, Milan, Italy;Univ Messina, Rheumatol Unit, Messina, Italy,University of Messina

Morel, J. (författare): Montpellier Univ Hosp, Dept Rheumatol, Montpellier, France;Univ Montpellier, Montpellier, France,University of Montpellier

Vollenveider, C. (författare): German Hosp, Buenos Aires, DF, Argentina,Hospital Aleman, Buenos Aires

Horvath, I-F (författare): University of Debrecen

Sivils, K. L. (författare): Oklahoma Med Res Fdn, Arthrit & Clin Immunol Res Program, 825 NE 13th St, Oklahoma City, OK 73104 USA,Oklahoma Medical Research Foundation,Hacettepe University,Hacettepe Univ, Fac Med, Dept Internal Med, Ankara, Turkey

Olsson, Peter (författare): Lund University,Lunds universitet,Internmedicin - epidemiologi,Forskargrupper vid Lunds universitet,Internal Medicine - Epidemiology,Lund University Research Groups,Skåne University Hospital,Lund Univ, Skane Univ Hosp Malmo, Dept Rheumatol, Malmo, Sweden

De Vita, S. (författare): Univ Hosp Santa Maria della Misericordia, Dept Med Area DAME, Clin Rheumatol, Udine, Italy,University Hospital 'Santa Maria della Misericordia'

Sanchez-Guerrero, J. (författare): Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Dept Rheumatol & Immunol, Mexico City, DF, Mexico,Salvador Zubirán National Institute of Health Sciences and Nutrition

Kilic, L. (författare): Hacettepe Univ, Fac Med, Dept Internal Med, Ankara, Turkey

Wahren-Herlenius, M. (författare): Karolinska Institute,Karolinska Institutet,Karolinska University Hospital,Karolinska Inst, Dept Med, Rheumatol Unit, Stockholm, Sweden;Karolinska Univ Hosp, Stockholm, Sweden

Mariette, X. (författare): Univ Paris Sud, Hop Univ Paris Sud, AP HP, Ctr Immunol Viral Infect & Autoimmune Dis,INSERM, Paris, France,University of Paris-Saclay

Ramos-Casals, M. (författare): Univ Barcelona, Sjogrens Syndrome Res Grp AGAUR, Lab Autoimmune Dis Josep Font,Hosp Clin, IDIBAPS,CELLEX,Dept Autoimmune Dis,ICMiD, Barcelona, Spain,University of Barcelona

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CLINICAL & EXPER RHEUMATOLOGY, 2018
2018
Engelska.
Ingår i: Clinical and Experimental Rheumatology. - : CLINICAL & EXPER RHEUMATOLOGY. - 0392-856X .- 1593-098X. ; 36:3, s. S102-S112

Relaterad länk:: http://www.clinexprh...; visa fler...; https://www.clinexpr...; https://urn.kb.se/re...; http://kipublication...; https://lup.lub.lu.s...; visa färre...

Tidskriftsartikel (refereegranskat)

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Objective: To evaluate the influence of the main immunological markers on the disease phenotype at diagnosis in a large international cohort of patients with primary Sjogren's syndrome (SjS).Methods: The Big Data Sjogren 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 cryoglobulinaemia correlated with systemic activity (mean ESSDAI score of 17.7 for ctyoglobulins, 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 ESSDAI evaluation were cryoglobulins (9 domains), low C3 (8 domains), anti-La (7 domains) and low C4 (6 domains).Conclusion: 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.

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Av författaren/redakt...: Brito-Zeron, P.; Acar-Denizli, N.; Ng, W. F.; Zeher, M.; Rasmussen, A.; Mandl, Thomas; visa fler...; Seror, R.; Li, X.; Baldini, C.; Gottenberg, J. - ...; Danda, D.; Quartuccio, L.; Priori, R.; Hernandez-Molina ...; Armagan, B.; Kruize, A. A.; Kwok, S. -K; Kvarnstrom, M.; Praprotnik, S.; Sene, D.; Bartoloni, E.; Solans, R.; Rischmueller, M.; Suzuki, Y.; Isenberg, D. A.; Valim, V.; Wiland, P.; Nordmark, Gunnel; Fraile, G.; Bootsma, H.; Nakamura, T.; Giacomelli, R.; Devauchelle-Pens ...; Knopf, A.; Bombardieri, M.; Trevisani, V. -F; Hammenfors, D.; Pasoto, S. G.; Retamozo, S.; Gheita, T. A.; Atzeni, F.; Morel, J.; Vollenveider, C.; Horvath, I-F; Sivils, K. L.; Olsson, Peter; De Vita, S.; Sanchez-Guerrero ...; Kilic, L.; Wahren-Herlenius ...; Mariette, X.; Ramos-Casals, M.; visa färre...

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Av lärosätet: Uppsala universitet; Karolinska Institutet; Lunds universitet

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How immunological profile drives clinical phenotype of primary Sjögren's syndrome at diagnosis : analysis of 10,500 patients (Sjögren Big Data Project)

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