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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
  1. Pilar Brito-Zerón1,2,
  2. Nihan Acar-Denizli3,
  3. Margit Zeher4,
  4. Astrid Rasmussen5,
  5. Raphaele Seror6,
  6. Elke Theander7,
  7. Xiaomei Li8,
  8. Chiara Baldini9,
  9. Jacques-Eric Gottenberg10,
  10. Debashish Danda11,
  11. Luca Quartuccio12,
  12. Roberta Priori13,
  13. Gabriela Hernandez-Molina14,
  14. Aike A Kruize15,
  15. Valeria Valim16,
  16. Marika Kvarnstrom17,
  17. Damien Sene18,
  18. Roberto Gerli19,
  19. Sonja Praprotnik20,
  20. David Isenberg21,
  21. Roser Solans22,
  22. Maureen Rischmueller23,
  23. Seung-Ki Kwok24,
  24. Gunnel Nordmark25,
  25. Yasunori Suzuki26,
  26. Roberto Giacomelli27,
  27. Valerie Devauchelle-Pensec28,
  28. Michele Bombardieri29,
  29. Benedikt Hofauer30,
  30. Hendrika Bootsma31,
  31. Johan G Brun32,
  32. Guadalupe Fraile33,
  33. Steven E Carsons34,
  34. Tamer A Gheita35,
  35. Jacques Morel36,
  36. Cristina Vollenveider37,
  37. Fabiola Atzeni38,
  38. Soledad Retamozo39,
  39. Ildiko Fanny Horvath4,
  40. Kathy Sivils5,
  41. Thomas Mandl7,
  42. Pulukool Sandhya11,
  43. Salvatore De Vita12,
  44. Jorge Sanchez-Guerrero14,
  45. Eefje van der Heijden15,
  46. Virginia Fernandes Moça Trevisani40,
  47. Marie Wahren-Herlenius17,
  48. Xavier Mariette6,
  49. Manuel Ramos-Casals2,41
  50. on behalf of the EULAR-SS Task Force Big Data Consortium
    1. 1Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona, Spain
    2. 2Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Department of Autoimmune Diseases, ICMiD, University of Barcelona, Hospital Clínic, Barcelona, Spain
    3. 3Department of Statistics, Faculty of Science and Letters, Mimar Sinan Fine Arts University, Istanbul, Turkey
    4. 4Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary
    5. 5Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA
    6. 6Center 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
    7. 7Department of Rheumatology, Malmö University Hospital, Lund University, Lund, Sweden
    8. 8Department of Rheumatology and Immunology, Anhui Provincial Hospital, Hefei, China
    9. 9Rheumatology Unit, University of Pisa, Pisa, Italy
    10. 10Department of Rheumatology, Strasbourg University Hospital, Université de Strasbourg, CNRS, Strasbourg, France
    11. 11Department of Clinical Immunology & Rheumatology, Christian Medical College & Hospital, Vellore, India
    12. 12Clinic of Rheumatology, Department of Medical and Biological Sciences, University Hospital “Santa Maria della Misericordia”, Udine, Italy
    13. 13Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Rome, Italy
    14. 14Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. México City, Mexico
    15. 15Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands
    16. 16Department of Medicine, Federal University of Espírito Santo, Vitória, Brazil
    17. 17Department of Medicine, Solna, Unit of Experimental Rheumatology, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden
    18. 18Département de Médecine Interne, Hôpital Lariboisière, Université Paris VII, Assistance Publique-Hôpitaux de Paris, Paris, France
    19. 19Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy
    20. 20Department of Rheumatology, University Medical Centre, Ljubljana, Slovenia
    21. 21Division of Medicine, Centre for Rheumatology, University College London, London, UK
    22. 22Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain
    23. 23Department of Rheumatology, The Queen Elizabeth Hospital, Discipline of Medicine University of Adelaide, South Australia
    24. 24Division of Rheumatology, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, South Korea
    25. 25Rheumatology, Department of Medical Sciences, Uppsala University, Uppsala, Sweden
    26. 26Division of Rheumatology, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan
    27. 27Clinical Unit of Rheumatology, School of Medicine, University of l'Aquila, L'Aquila, Italy
    28. 28Rheumatology Department, Brest University Hospital, Brest, France
    29. 29Centre for Experimental Medicine and Rheumatology, Queen Mary University of London, London, UK
    30. 30Otorhinolaryngology / Head and Neck Surgery, Technical University Munich, Munich, Germany
    31. 31Department of Rheumatology & Clinical Immunology, University of Groningen, University Medical Center Groningen, Groningen, The Netherlands
    32. 32Department of Clinical Science, University of Bergen; and Department of Rheumatology, Haukeland University Hospital, Bergen, Norway
    33. 33Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain
    34. 34Division of Rheumatology, Allergy and Immunology Winthrop-University Hospital, Stony Brook University School of Medicine, Mineola, New York, USA
    35. 35Rheumatology Department, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt
    36. 36Department of Rheumatology, Teaching hospital and University of Montpellier, Montpellier, France
    37. 37German Hospital, Buenos Aires, Argentina
    38. 38IRCCS Galeazzi Orthopedic Institute, Milan, Italy
    39. 39Hospital Privado Universitario de Córdoba, Institute University of Biomedical Sciences University of Córdoba (IUCBC), Cordoba, Argentina
    40. 40Federal University of São Paulo, Sao Paulo, Brazil
    41. 41Department of Medicine, University of Barcelona, Barcelona, Spain
    1. Correspondence to Dr Manuel Ramos-Casals, Servei de Malalties Autoimmunes Sistèmiques, Hospital Clínic, C/Villarroel, 170, Barcelona 08036, Spain; mramos{at}clinic.ub.es

    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.

    • Sjøgren's Syndrome
    • Autoimmune Diseases
    • Autoimmunity
    • Epidemiology

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    Footnotes

    • Handling editor Tore K Kvien

    • Collaborators Appendix 1 Members of the European League Against Rheumatism (EULAR)-SS Task Force Big Data Consortium: (A) members of the EULAR-SS Task Force: P Brito-Zeróna and C Morcillo (Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona, Spain); P Brito-Zerón, I García-Sánchez, H Gheitasi, A Bové, M Ramos-Casals (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); N Acar-Denizli (Department of Statistics, Faculty of Science and Letters, Mimar Sinan Fine Arts University, Istanbul, Turkey); M Zeher, Ildike-Fanny Horvath (Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary); A Rasmussen, K Sivils and H Scofield (Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, Oklahoma, USA); R Seror and X Mariette (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, Paris, France Paris, France); E Theander and T Mandl (Department of Rheumatology, Malmö University Hospital, Lund University, Lund, Sweden); X Li (Department of Rheumatology and Immunology, Anhui Provincial Hospital, China); C Baldini (Rheumatology Unit, University of Pisa, Pisa, Italy); JE Gottenberg (Department of Rheumatology, Strasbourg University Hospital, Université de Strasbourg, CNRS, Strasbourg, France); D Danda and P Sandhya (Department of Clinical Immunology and Rheumatology, Christian Medical College and Hospital, Vellore, India); L Quartuccio, L Corazza and S De Vita (Clinic of Rheumatology, Department of Medical and Biological Sciences, University Hospital “Santa Maria della Misericordia”, Udine, Italy); R Priori (Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Italy); G Hernandez-Molina and J Sánchez-Guerrero (Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. México City, Mexico); AA Kruize and E van der Heijden (Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands); V Valim (Department of Medicine, Federal University of Espírito Santo, Vitória, Brazil); M Kvarnstrom and M Wahren-Herlenius (Department of Medicine, Solna, Unit of Experimental Rheumatology, Karolinska Institutet and Karolinska University Hospital, Stockholm); D Sene (Service de Médecine Interne 2, Hôpital Lariboisière, Université Paris VII, Assistance Publique-Hôpitaux de Paris, 2, Paris, France); R Gerli and E Bartoloni (Rheumatology Unit, Department of Medicine, University of Perugia, Italy); S Praprotnik (Department of Rheumatology, University Medical Centre, Ljubljana, Slovenia); D Isenberg (Centre for Rheumatology, Division of Medicine, University College London, UK); R Solans (Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain); M Rischmueller and S Downie-Doyle (Department of Rheumatology, School of Medicine, The University of Western Australia, Crawley, Australia); S-K Kwok and S-H Park (Seoul St Mary's Hospital, The Catholic University of Korea, Seoul); G Nordmark (Rheumatology, Department of Medical Sciences, Uppsala University, Uppsala, Sweden); Y Suzuki and M Kawano (Division of Rheumatology, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan); R Giacomelli and F Carubbi (Clinical Unit of Rheumatology, University of l'Aquila, School of Medicine, L'Aquila, Italy); V Devauchelle-Pensec and A Saraux (Rheumatology Department, Brest University Hospital, Brest, France); M Bombardieri and E Astorri (Centre for Experimental Medicine and Rheumatology, Queen Mary University of London, UK); B Hofauer (Hals-Nasen-Ohrenklinik und Poliklinik, Technische Universität München, München, Germany); H Bootsma and A Vissink (Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Center Groningen, the Netherlands); JG Brun and D Hammenfors (Department of Rheumatology, Haukeland University Hospital, Bergen, Norway); G Fraile (Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain); SE Carsons (Division of Rheumatology, Allergy and Immunology Winthrop-University Hospital, Stony Brook University School of Medicine, Mineola, New York, USA); TA Gheita, (Rheumatology Department, Kasr Al Ainy School of Medicine, Cairo University, Egypt); HM Khalil (Ophthalmology Department, Faculty of Medicine, Beni Suef University, Egypt); J Morel (Department of Rheumatology, Teaching hospital and University of Montpellier, Montpellier, France); C Vollenveider (German Hospital, Buenos Aires, Argentina); F Atzeni (IRCCS Galeazzi Orthopedic Institute, Milan, Italy); S Retamozo (Hospital Privado Universitario de Córdoba, Institute University of Biomedical Sciences University of Córdoba, Córdoba, Argentina); V Moça Trevisano (Federal University of São Paulo, Sao Paulo, Brazil); B Kostov and A Sisó-Almirall (Primary Care Research Group, IDIBAPS, Centre d'Assistència Primària ABS Les Corts, CAPSE, Barcelona, Spain). (B) Members of the French ASSESS Cohort: J Sibilia (Rheumatology Centre National de Référence des Maladies Auto-Immunes Rares, Institut National de la Santé et de la Recherche Médicale UMRS_1109, Fédération de Médecine Translationnelle de Strasbourg, Strasbourg University Hospital, Université de Strasbourg, Strasbourg, France); C Miceli-Richard and G Nocturne (Rheumatology, Bicetre Hospital, Institut National de la Santé et de la Recherche Médicale U-1012, Université Paris Sud, Assistance Publique des Hôpitaux de Paris, Paris, France); J Benessiano (Centre de Ressources Biologiques, Bichat Hospital, Assistance Publique des Hôpitaux de Paris, Paris, France); P Dieude (Rheumatology, Bichat Hospital, Assistance Publique des Hôpitaux de Paris, Paris, France); J-J Dubost (Rheumatology, Clermont-Ferrand Hospital, Clermont-Ferrand, France); A-L Fauchais (Internal Medicine, Limoges Hospital, Limoges, France); V Goeb (Rheumatology, Amiens University Hospital, Amiens, France); E Hachulla (Pierre Yves Hatron, Internal Medicine, Lille University Hospital, Lille, France); C Larroche (Internal Medicine, Avicenne Hospital, Assistance Publique des Hôpitaux de Paris, Bobigny, France); V Le Guern and X Puéchal (Internal Medicine, Cochin Hospital, Assistance Publique des Hôpitaux de Paris, Paris, France); J Morel (Rheumatology, Montpellier University Hospital, Montpellier, France); A Perdriger (Rheumatology, Rennes University Hospital, Rennes, France); S Rist (Rheumatology, Orléans Hospital, Orléans, France); O Vittecoq (Rheumatology, Rouen University Hospital, Rouen, France); P Ravaud (Centre of Clinical Epidemiology, Hotel Dieu Hospital, Assistance Publique des Hôpitaux de Paris, Institut National de la Santé et de la Recherche Médicale U378, University of Paris Descartes, Faculty of Medicine, Paris, France). (C) Members of the Spanish GEAS Cohort (SS Study Group, Autoimmune Diseases Study Group GEAS, Spanish Society of Internal Medicine SEMI): B Díaz-López (Department of Internal Medicine, Hospital Universitario Central de Asturias, Oviedo, Spain); A Casanovas, (Department of Internal Medicine, Hospital Parc Taulí, Sabadell, Spain); L Pallarés (Department of Internal Medicine, Hospital Son Espases, Palma de Mallorca, Spain); M López-Dupla (Department of Internal Medicine, Hospital Joan XXIII, Tarragona, Spain); R Pérez-Alvarez (Department of Internal Medicine, Hospital do Meixoeiro, Vigo, Spain); M Ripoll (Department of Internal Medicine, Hospital Infanta Sofía, Madrid, Spain); B Pinilla (Department of Internal Medicine, Hospital Gregorio Marañón, Madrid, Spain); M Akasbi (Department of Internal Medicine, Hospital Infanta Leonor, Madrid, Spain); B Maure (Department of Internal Medicine, Complejo Hospitalario Universitario, Vigo, Spain); E Fonseca (Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain); J Canora (Department of Internal Medicine, Hospital Universitario de Fuenlabrada, Madrid, Spain); G de la Red (Department of Internal Medicine, Hospital Espíritu Santo, Barcelona, Spain); AJ Chamorro (Department of Internal Medicine, Complejo Hospitalario de Ourense, Ourense, Spain); I Jiménez-Heredia (Department of Internal Medicine, Hospital de Manises, Valencia, Spain); P Fanlo (Complejo Universitario de Navarra, Spain); P Guisado-Vasco (Hospital Quirón, Madrid, Spain) and M Zamora (Hospital Virgen de las Nieves, Granada, Spain).

    • Contributors Conception and design: PB-Z and MR-C; acquisition of data: all authors; analysis and interpretation of data: PB-Z, NA-D and MR-C; statistical analysis: NA-D and MR-C; drafting the article or revising it critically for important intellectual content: all authors; final approval of the version published: all authors.

    • Funding Supported by Grants Fondo de Investigaciones Sanitarias (MRC, INT15/00085) and ‘Ajut per a la Recerca Josep Font’ (PBZ, Hospital Clinic-Barcelona 2012).

    • Competing interests None declared.

    • Patient consent Obtained.

    • Ethics approval Committee of the Coordinating Centre (Hospital Clinic, Barcelona, Spain, registry HCB/2015/0869).

    • Provenance and peer review Not commissioned; externally peer reviewed.

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