Influence of geolocation and ethnicity on the phenotypic expression of primary Sjögren's syndrome at diagnosis in 8310 patients: a cross-sectional study from the Big Data Sjögren Project Consortium.

Pilar Brito-Zerón, Nihan Acar-Denizli, Margit Zeher, Astrid Rasmussen, Raphaele Seror, Elke Theander, Xiaomei Li, Chiara Baldini, Jacques-Eric Gottenberg, Debashish Danda, Luca Quartuccio, Roberta Priori, Gabriela Hernandez-Molina, Aike A Kruize, Valeria Valim, Marika Kvarnstrom, Damien Sene, Roberto Gerli, Sonja Praprotnik, David Isenberg, Roser Solans, Maureen Rischmueller, Seung-Ki Kwok, Gunnel Nordmark, Yasunori Suzuki, Roberto Giacomelli, Valerie Devauchelle-Pensec, Michele Bombardieri, Benedikt Hofauer, Hendrika Bootsma, Johan G Brun, Guadalupe Fraile, Steven E Carsons, Tamer A Gheita, Jacques Morel, Cristina Vollenveider, Fabiola Atzeni, Soledad Retamozo, Ildiko Fanny Horvath, Kathy Sivils, Thomas Mandl, Pulukool Sandhya, Salvatore De Vita, Jorge Sanchez-Guerrero, Eefje van der Heijden, Virginia Fernandes Moça Trevisani, Marie Wahren-Herlenius, Xavier Mariette, Manuel Ramos-Casals

Journal: Annals of the rheumatic diseases 2017;76(6):1042-1050

PMID: 27899373

Abstract

OBJECTIVES

To analyse the influence of geolocation and ethnicity on the clinical presentation of primary Sjögren's syndrome (SjS) at diagnosis.

METHODS

The Big Data Sjögren Project Consortium is an international, multicentre registry designed in 2014. By January 2016, 20 centres from five continents were participating. Multivariable logistic regression analyses were performed.

RESULTS

We included 7748 women (93%) and 562 men (7%), with a mean age at diagnosis of primary SjS of 53 years. Ethnicity data were available for 7884 patients (95%): 6174 patients (78%) were white, 1066 patients (14%) were Asian, 393 patients (5%) were Hispanic, 104 patients (1%) were black/African-American and 147 patients (2%) were of other ethnicities. SjS was diagnosed a mean of 7 years earlier in black/African-American compared with white patients; the female-to-male ratio was highest in Asian patients (27:1) and lowest in black/African-American patients (7:1); the prevalence of sicca symptoms was lowest in Asian patients; a higher frequency of positive salivary biopsy was found in Hispanic and white patients. A north-south gradient was found with respect to a lower frequency of ocular involvement in northern countries for dry eyes and abnormal ocular tests in Europe (OR 0.46 and 0.44, respectively) and Asia (OR 0.18 and 0.49, respectively) compared with southern countries. Higher frequencies of antinuclear antibodies (ANAs) were reported in northern countries in America (OR=1.48) and Asia (OR=3.80) while, in Europe, northern countries had lowest frequencies of ANAs (OR=0.67) and Ro/La (OR=0.69).

CONCLUSIONS

This study provides the first evidence of a strong influence of geolocation and ethnicity on the phenotype of primary SjS at diagnosis.

Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

Address: Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona, Spain.; Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Department of Autoimmune Diseases, ICMiD, University of Barcelona, Hospital Clínic, Barcelona, Spain.; Department of Statistics, Faculty of Science and Letters, Mimar Sinan Fine Arts University, Istanbul, Turkey.; Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary.; Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA.; Center for Immunology of Viral Infections and Autoimmune Diseases, Assistance Publique-Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, Le Kremlin-Bicêtre, Université Paris Sud, INSERM U1184, Paris, France.; Department of Rheumatology, Malmö University Hospital, Lund University, Lund, Sweden.; Department of Rheumatology and Immunology, Anhui Provincial Hospital, Hefei, China.; Rheumatology Unit, University of Pisa, Pisa, Italy.; Department of Rheumatology, Strasbourg University Hospital, Université de Strasbourg, CNRS, Strasbourg, France.; Department of Clinical Immunology & Rheumatology, Christian Medical College & Hospital, Vellore, India.; Clinic of Rheumatology, Department of Medical and Biological Sciences, University Hospital "Santa Maria della Misericordia", Udine, Italy.; Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Rome, Italy.; Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. México City, Mexico.; Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.; Department of Medicine, Federal University of Espírito Santo, Vitória, Brazil.; Department of Medicine, Solna, Unit of Experimental Rheumatology, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden.; Département de Médecine Interne, Hôpital Lariboisière, Université Paris VII, Assistance Publique-Hôpitaux de Paris, Paris, France.; Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy.; Department of Rheumatology, University Medical Centre, Ljubljana, Slovenia.; Division of Medicine, Centre for Rheumatology, University College London, London, UK.; Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain.; Department of Rheumatology, The Queen Elizabeth Hospital, Discipline of Medicine University of Adelaide, South Australia.; Division of Rheumatology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, South Korea.; Rheumatology, Department of Medical Sciences, Uppsala University, Uppsala, Sweden.; Division of Rheumatology, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan.; Clinical Unit of Rheumatology, School of Medicine, University of l'Aquila, L'Aquila, Italy.; Rheumatology Department, Brest University Hospital, Brest, France.; Centre for Experimental Medicine and Rheumatology, Queen Mary University of London, London, UK.; Otorhinolaryngology / Head and Neck Surgery, Technical University Munich, Munich, Germany.; Department of Rheumatology & Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands.; Department of Clinical Science, University of Bergen; and Department of Rheumatology, Haukeland University Hospital, Bergen, Norway.; Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain.; Division of Rheumatology, Allergy and Immunology Winthrop-University Hospital, Stony Brook University School of Medicine, Mineola, New York, USA.; Rheumatology Department, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt.; Department of Rheumatology, Teaching hospital and University of Montpellier, Montpellier, France.; German Hospital, Buenos Aires, Argentina.; IRCCS Galeazzi Orthopedic Institute, Milan, Italy.; Hospital Privado Universitario de Córdoba, Institute University of Biomedical Sciences University of Córdoba (IUCBC), Cordoba, Argentina.; Federal University of São Paulo, Sao Paulo, Brazil.; Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Department of Autoimmune Diseases, ICMiD, University of Barcelona, Hospital Clínic, Barcelona, Spain.; Department of Medicine, University of Barcelona, Barcelona, Spain.
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