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  • Brito-Zeron, P.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 (author)

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

  • Article/chapterEnglish2018

Publisher, publication year, extent ...

  • CLINICAL & EXPER RHEUMATOLOGY,2018
  • printrdacarrier

Numbers

  • LIBRIS-ID:oai:DiVA.org:uu-368779
  • https://urn.kb.se/resolve?urn=urn:nbn:se:uu:diva-368779URI
  • http://kipublications.ki.se/Default.aspx?queryparsed=id:139348478URI
  • https://lup.lub.lu.se/record/d6ac6b68-a4bb-42ee-ae4a-8d8e298e74f3URI

Supplementary language notes

  • Language:English
  • Summary in:English

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  • Subject category:ref swepub-contenttype
  • Subject category:art swepub-publicationtype

Notes

  • 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.

Subject headings and genre

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  • Acar-Denizli, N.Mimar Sinan Fine Arts Univ, Fac Sci & Letters, Dept Stat, Istanbul, Turkey,Mimar Sinan Fine Arts University (author)
  • Ng, W. F.Newcastle Univ, Inst Cellular Med, Newcastle Upon Tyne, Tyne & Wear, England,University of Newcastle upon Tyne (author)
  • Zeher, M.Univ Debrecen, Fac Med, Div Clin Immunol, Debrecen, Hungary,University of Debrecen (author)
  • Rasmussen, A.Oklahoma Med Res Fdn, Arthrit & Clin Immunol Res Program, 825 NE 13th St, Oklahoma City, OK 73104 USA,Oklahoma Medical Research Foundation (author)
  • Mandl, ThomasLund 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(Swepub:lu)medf-tma (author)
  • Seror, R.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 (author)
  • Li, X.Anhui Prov Hosp, Dept Rheumatol & Immunol, Hefei, Anhui, Peoples R China,Anhui Provincial Hospital (author)
  • Baldini, C.Univ Pisa, Rheumatol Unit, Pisa, Italy,University of Pisa (author)
  • Gottenberg, J. -EUniversity of Strasbourg (author)
  • Danda, D.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 (author)
  • Quartuccio, L.Univ Hosp Santa Maria della Misericordia, Dept Med Area DAME, Clin Rheumatol, Udine, Italy,University Hospital 'Santa Maria della Misericordia' (author)
  • Priori, R.Sapienza Univ Rome, Rheumatol Clin, Dept Internal Med & Med Specialties, Rome, Italy,Sapienza University of Rome (author)
  • Hernandez-Molina, G.Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Dept Rheumatol & Immunol, Mexico City, DF, Mexico,Salvador Zubirán National Institute of Health Sciences and Nutrition (author)
  • Armagan, B.Hacettepe Univ, Fac Med, Dept Internal Med, Ankara, Turkey,Hacettepe University (author)
  • Kruize, A. A.Univ Med Ctr Utrecht, Dept Rheumatol & Clin Immunol, Utrecht, Netherlands,University Medical Center Utrecht (author)
  • Kwok, S. -KCatholic University of Korea,Oklahoma Med Res Fdn, Arthrit & Clin Immunol Res Program, 825 NE 13th St, Oklahoma City, OK 73104 USA (author)
  • Kvarnstrom, M.Karolinska Institute,Karolinska Institutet,Karolinska University Hospital,Karolinska Inst, Dept Med, Rheumatol Unit, Stockholm, Sweden;Karolinska Univ Hosp, Stockholm, Sweden (author)
  • Praprotnik, S.Univ Med Ctr, Dept Rheumatol, Ljubljana, Slovenia,University Medical Centre Ljubljana (author)
  • Sene, D.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 (author)
  • Bartoloni, E.Univ Perugia, Dept Med, Rheumatol Unit, Perugia, Italy,University of Perugia (author)
  • Solans, R.Hosp Valle De Hebron, Dept Internal Med, Barcelona, Spain,Vall d'Hebron University Hospital (author)
  • Rischmueller, M.Univ Western Australia, Sch Med, Dept Rheumatol, Crawley, Australia,University of Western Australia, Crawley (author)
  • Suzuki, Y.Kanazawa Univ Hosp, Div Rheumatol, Kanazawa, Ishikawa, Japan,Kanazawa University Hospital (author)
  • Isenberg, D. A.UCL, Div Med, Ctr Rheumatol, London, England,University College London (author)
  • Valim, V.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 (author)
  • Wiland, P.Wroclaw Med Hosp, Dept Rheumatol & Internal Med, Wroclaw, Poland,Wroclaw Medical Hospital (author)
  • Nordmark, GunnelUppsala University,Uppsala universitet,Reumatologi(Swepub:uu)gunnnord (author)
  • Fraile, G.Hosp Ramon & Cajal, Dept Internal Med, Madrid, Spain,Hospital Ramón Y Cajal (author)
  • Bootsma, H.Univ Groningen, Univ Med Ctr Groningen, Dept Rheumatol & Clin Immunol, Groningen, Netherlands,University of Groningen (author)
  • Nakamura, T.Nagasaki Univ, Grad Sch Biomed Sci, Dept Radiol & Canc Biol, Nagasaki, Japan,Nagasaki University (author)
  • Giacomelli, R.Univ Aquila, Sch Med, Clin Unit Rheumatol, Laquila, Italy,University of L'Aquila (author)
  • Devauchelle-Pensec, V.Brest Univ Hosp, Dept Rheumatol, Brest, France,University Hospital of Brest (author)
  • Knopf, A.Tech Univ Munich, Klinikum Rechts Isar, Otorhinolaryngol Head & Neck Surg, Munich, Germany,Technical University of Munich (author)
  • Bombardieri, M.Queen Mary Univ London, Ctr Expt Med & Rheumatol, London, England,Queen Mary University (author)
  • Trevisani, V. -FUniv Fed Sao Paulo, Sao Paulo, Brazil,Federal University of São Paulo (author)
  • Hammenfors, D.Univ Bergen, Dept Clin Sci, Bergen, Norway;Haukeland Hosp, Dept Rheumatol, Bergen, Norway,Haukeland University Hospital,University of Bergen (author)
  • Pasoto, S. G.Univ Sao Paulo, Fac Med, Hosp Clin, Div Rheumatol, Sao Paulo, Brazil,University of São Paulo (author)
  • Retamozo, S.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 (author)
  • Gheita, T. A.Cairo Univ, Kasr Al Ainy Sch Med, Dept Rheumatol, Cairo, Egypt,Cairo University (author)
  • Atzeni, F.IRCCS Galeazzi Orthopaed Inst, Milan, Italy;Univ Messina, Rheumatol Unit, Messina, Italy,University of Messina (author)
  • Morel, J.Montpellier Univ Hosp, Dept Rheumatol, Montpellier, France;Univ Montpellier, Montpellier, France,University of Montpellier (author)
  • Vollenveider, C.German Hosp, Buenos Aires, DF, Argentina,Hospital Aleman, Buenos Aires (author)
  • Horvath, I-FUniversity of Debrecen (author)
  • Sivils, K. L.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 (author)
  • Olsson, PeterLund 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(Swepub:lu)med-pol (author)
  • De Vita, S.Univ Hosp Santa Maria della Misericordia, Dept Med Area DAME, Clin Rheumatol, Udine, Italy,University Hospital 'Santa Maria della Misericordia' (author)
  • Sanchez-Guerrero, J.Inst Nacl Ciencias Med & Nutr Salvador Zubiran, Dept Rheumatol & Immunol, Mexico City, DF, Mexico,Salvador Zubirán National Institute of Health Sciences and Nutrition (author)
  • Kilic, L.Hacettepe Univ, Fac Med, Dept Internal Med, Ankara, Turkey (author)
  • Wahren-Herlenius, M.Karolinska Institute,Karolinska Institutet,Karolinska University Hospital,Karolinska Inst, Dept Med, Rheumatol Unit, Stockholm, Sweden;Karolinska Univ Hosp, Stockholm, Sweden (author)
  • Mariette, X.Univ Paris Sud, Hop Univ Paris Sud, AP HP, Ctr Immunol Viral Infect & Autoimmune Dis,INSERM, Paris, France,University of Paris-Saclay (author)
  • Ramos-Casals, M.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 (author)
  • 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, SpainHospital CIMA (creator_code:org_t)
  • Sjögren Big Data Consortium

Related titles

  • In:Clinical and Experimental Rheumatology: CLINICAL & EXPER RHEUMATOLOGY36:3, s. S102-S1120392-856X1593-098X

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