How immunological profile drives clinical phenotype of primary Sjogren's syndrome at diagnosis:analysis of 10,500 patients (Sjogren Big Data Project)
Year: 2018
Type: article
Study design: Observational Study
Abstract: 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 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.
Authors: Sjogren Big Data Consortium, P. Brito-Zeron, N. Acar-Denizli, W. F. Ng, M. Zeher, A. Rasmussen, T. Mandl, R. Seror, X. Li, C. Baldini, J. -E. Gottenberg, D. Danda, L. Quartuccio, R. Priori, G. Hernandez-Molina, B. Armagan, A. A. Kruize, S. -K. Kwok, M. Kvarnstrom, S. Praprotnik, D. Sene, E. Bartoloni, R. Solans, M. Rischmueller, Y. Suzuki, D. A. Isenberg, V. Valim, P. Wiland, G. Nordmark, G. Fraile, H. Bootsma, T. Nakamura, R. Giacomelli, V. Devauchelle-Pensec, A. Knopf, M. Bombardieri, V. -F Trevisani, D. Hammenfors, S. G. Pasoto, S. Retamozo, T. A. Gheita, F. Atzeni, J. Morel, C. Vollenveider, I-F. Horvath, K. L. Sivils, P. Olsson, S. De Vita, J. Sanchez-Guerrero, L. Kilic, M. Wahren-Herlenius
Institutions: Agència de Gestió d'Ajuts Universitaris i de Recerca, Consorci Institut D'Investigacions Biomediques August Pi I Sunyer, Hospital Sanitas CIMA, Universitat de Barcelona, Mimar Sinan Güzel Sanatlar Üniversitesi, Newcastle University, University of Debrecen, Oklahoma Medical Research Foundation, Lund University, Skåne University Hospital, Inserm, Assistance Publique – Hôpitaux de Paris, Bicêtre Hospital, Hôpitaux universitaires Paris Sud, Anhui Provincial Hospital, University of Pisa, Centre National de la Recherche Scientifique, Hôpitaux Universitaires de Strasbourg, Université de Strasbourg, Christian Medical College, Vellore, Sapienza University of Rome, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, Hacettepe University, University Medical Center Utrecht, The Catholic University of Korea Seoul St. Mary's Hospital, Catholic University of Korea, Karolinska University Hospital, Karolinska Institutet, Ljubljana University Medical Centre, Hôpital Lariboisière, Université Paris Diderot, University of Perugia, Vall d'Hebron Hospital Universitari, The University of Western Australia, Kanazawa University, Kanazawa University Hospital, University of London, University College London, Universidade Federal do Espírito Santo, Wroclaw Medical University, Uppsala University, Instituto Ramón y Cajal de Investigación Sanitaria, Hospital Universitario Ramón y Cajal, Nagasaki University, University of L'Aquila, Université Bretagne Loire, TUM Klinikum, Technical University of Munich, Queen Mary University of London, William Harvey Research Institute, Universidade Federal de São Paulo, Haukeland University Hospital, University of Bergen, Universidade de São Paulo, Consejo Nacional de Investigaciones Científicas y Técnicas, Instituto de Investigaciones en Ciencias de la Salud, Cairo University, University of Messina, IRCCS Ospedale Galeazzi - Sant'Ambrogio, Université de Montpellier, COMUE Languedoc-Roussillon Universités, Centre Hospitalier Universitaire de Montpellier, Universidad de Buenos Aires, Hospital Alemán
Language: English
Subfield: Physiology (medical)
Field: Medicine
Domain: Health Sciences
Open Access status: green
Funders: University College London, Institut National de la Santé et de la Recherche Médicale, Lunds Universitet, Université de Strasbourg, Karolinska Institutet, Sapienza Università di Roma, Centre National de la Recherche Scientifique, Malmö Högskola, Università di Pisa, Université de Montpellier, Assistance publique-Hôpitaux de Paris, Università degli Studi di Perugia, Universidade Federal do Espírito Santo, Directorate for Biological Sciences
OpenAlex ID: W4213466126