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Clinical aspects

 

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

1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11, 12, 13, 14, 15, 16, 17, 18, 19, 20, 21, 22, 23, 24, 25, 26, 27, 28, 29, 30, 31, 32, 33, 34, 35, 36, 37, 38, 39, 40, 41, 42, 43, 44, 45, 46, 47, 48, 49, 50, 51, 52

 

  1. Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona, Spain.
  2. Department of Statistics, Faculty of Science and Letters, Mimar Sinan Fine Arts University, Istanbul, Turkey.
  3. Institute of Cellular Medicine, Newcastle University, Newcastle Upon Tyne, UK.
  4. Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Debrecen, Hungary.
  5. Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, USA.
  6. Department of Rheumatology, Skane University Hospital Malmö, Lund University, Malmö, Sweden.
  7. 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.
  8. Department of Rheumatology and Immunology, Anhui Provincial Hospital, Hefei, China.
  9. Rheumatology Unit, University of Pisa, Italy.
  10. Department of Rheumatology, Strasbourg University Hospital, Université de Strasbourg, CNRS, Strasbourg, France.
  11. Department of Clinical Immunology & Rheumatology, Christian Medical College & Hospital, Vellore, India.
  12. Clinic of Rheumatology, Department of Medical Area (DAME), University Hospital 'Santa Maria della Misericordia', Udine, Italy.
  13. Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Italy.
  14. Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. México City, Mexico.
  15. Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
  16. Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
  17. Seoul St. Mary's Hospital, The Catholic University of Korea, Seoul, South Korea.
  18. Department of Medicine, Solna, Unit of Rheumatology, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden.
  19. Department of Rheumatology, University Medical Centre, Ljubljana, Slovenia
  20. Department of Internal Medicine, Lariboisière Hospital, Assistance Publique-Hôpitaux de Paris , Paris Diderot University, Paris, France.
  21. Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy.
  22. Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain.
  23. Department of Rheumatology, School of Medicine, The University of Western Australia, Crawley, Australia.
  24. Division of Rheumatology , Kanazawa University Hospital, Kanazawa, Ishikawa, Japan.
  25. Centre for Rheumatology, Division of Medicine , University College London, London, UK.
  26. Department of Medicine, Federal University of Espírito Santo and University Hospital HUCAM/EBSERH, Vitória, Brazil.
  27. Department of Rheumatology and Internal Medicine, Wroclaw Medical Hospital, Wroclaw, Poland
  28. Rheumatology, Department of Medical Sciences, Uppsala University, Uppsala, Sweden.
  29. Department of Internal Medicine, Hospital Ramón y Cajal, Madrid, Spain.
  30. Department of Rheumatology & Clinical Immunology, University of Groningen, University Medical Center Groningen, The Netherlands.
  31. Department of Radiology and Cancer Biology, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan
  32. Clinical Unit of Rheumatology, University of l'Aquila, School of Medicine, L'Aquila, Italy.
  33. Rheumatology Department, Brest University Hospital, Brest, France.
  34. Otorhinolaryngology/Head and Neck Surgery, Klinikum rechts der Isar, Technical University Munich, Germany.
  35. Centre for Experimental Medicine and Rheumatology, Queen Mary University of London, UK.
  36. Federal University of São Paulo, Sao Paulo, Brazil.
  37. Department of Clinical Science, University of Bergen; and Department of Rheumatology, Haukeland University Hospital, Bergen, Norway.
  38. Rheumatology Division, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Brazil.
  39. Hospital Privado Universitario de Córdoba, Instituto Universitario de Ciencias Biomédicas de Córdoba, Instituto De Investigaciones En Ciencias De La Salud, Univ. Nacional de Córdoba, Consejo Nacional de Investigaciones Científicas y Técnicas, Argentina.
  40. Rheumatology Department, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt.
  41. IRCCS Galeazzi Orthopedic Institute, Milan, and Rheumatology Unit, University of Messina, Italy.
  42. Department of Rheumatology, Montpellier University Hospital and University of Montpellier, Montpellier, France.
  43. German Hospital, Buenos Aires, Argentina.
  44. Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Hungary.
  45. Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, USA.
  46. Department of Rheumatology, Skane University Hospital Malmö, Lund University, Malmö, Sweden.
  47. Clinic of Rheumatology, Department of Medical Area (DAME), University Hospital 'Santa Maria della Misericordia', Udine, Italy.
  48. Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán. México City, Mexico.
  49. Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
  50. Department of Medicine, Solna, Unit of Rheumatology, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden.
  51. 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.
  52. 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. mramos@clinic.ub.es

on behalf of the Sjögren Big Data Consortium

CER11329
2018 Vol.36, N°3 ,Suppl.112
PI 0102, PF 0112
Clinical aspects

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PMID: 30156539 [PubMed]

Received: 17/04/2018
Accepted : 17/05/2018
In Press: 14/08/2018
Published: 14/08/2018

Abstract

OBJECTIVES:
To evaluate the influence of the main immunological markers on the disease phenotype at diagnosis in a large international cohort of patients with primary Sjögren’s syndrome (SjS).
METHODS:
The Big Data Sjögren 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 cryoglo-bulinaemia correlated with systemic activity (mean ESSDAI score of 17.7 for cryoglobulins, 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 ESS- DAI evaluation were cryoglobulins (9 domains), low C3 (8 domains), anti-La (7 domains) and low C4 (6 domains).
CONCLUSIONS:
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.

Rheumatology Article