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Influence of the age at diagnosis in the disease expression of primary Sjögren syndrome. Analysis of 12,753 patients from the Sjögren Big Data Consortium


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, 53, 54, 55, 56, 57, 58, 59, 60, 61, 62, 63, 64, 65, 66, 67, 68, 69, 70, 71

 

  1. Instituto Modelo de Cardiología Privado SRL, Córdoba, and Instituto Universitario de Ciencias Biomédicas de Córdoba (IUCBC), Argentina; and Rheumatology Department, Hospital Universitari Parc Taulí, Sabadell, Barcelona, Spain.
  2. Department of Statistics and Operations Research, Universitat Politècnica de Catalunya, Barcelona, Spain.
  3. Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Hungary.
  4. Institute of Cellular Medicine, Newcastle University, and NIHR Biomedical Research Centre, Newcastle Upon Tyne, UK.
  5. Genes and Human Disease Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, USA.
  6. Department of Rheumatology, Peking Union Medical College Hospital, Beijing, China.
  7. Department of Rheumatology and Immunology, Anhui Provincial Hospital, Hefei, China.
  8. Rheumatology Unit, University of Pisa, Italy.
  9. Department of Rheumatology, Skane University Hospital Malmö, Lund University, Sweden.
  10. Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, and Saint Camillus International University of Health Science, UniCamillus, Rome, Italy.
  11. Centre 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.
  12. Department of Rheumatology, Strasbourg University Hospital, Université de Strasbourg, CNRS, Strasbourg, France.
  13. Department of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, The Netherlands.
  14. Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, México City, Mexico.
  15. Department of Oral and Maxillofacial Surgery, University of Groningen, University Medical Centre Groningen, the Netherlands.
  16. Department of Clinical Immunology and Rheumatology, Christian Medical College and Hospital, Vellore, India.
  17. Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
  18. Clinic of Rheumatology, Department of Medical and Biological Sciences, University Hospital "Santa Maria della Misericordia", Udine, Italy.
  19. Department of Rheumatology and Internal Medicine, Wroclaw Medical Hospital, Wroclaw, Poland.
  20. Department of Rheumatology, University Medical Centre, Ljubljana, Slovenia.
  21. Rheumatology Unit, Department of Medicine, University of Perugia, Italy.
  22. Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, South Korea.
  23. Department of Medicine, Solna, Division of Experimental Rheumatology, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden.
  24. Department of Rheumatology, The Queen Elizabeth Hospital, Discipline of Medicine, University of Adelaide, South Australia, Australia.
  25. Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain.
  26. Service de Médecine Interne 2, Hôpital Lariboisière, Université Paris VII, Assistance Publique-Hôpitaux de Paris 2, Paris, France.
  27. Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina da Universidade de Sao Paulo, Brazil.
  28. Division of Rheumatology, Kanazawa University Hospital, Kanazawa, Ishikawa, Japan.
  29. Centre for Rheumatology, Division of Medicine, University College London, UK.
  30. Department of Medicine, Federal University of Espírito Santo, Vitória, Brazil.
  31. Rheumatology, Department of Medical Sciences, University of Uppsala, Sweden.
  32. Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
  33. Division of Health Based Evidence, Federal University of São Paulo, Brazil.
  34. Otorhinolaryngology, Head and Neck Surgery, Technical University Munich, Germany.
  35. Primary Care Centre Les Corts, Consorci d’Atenció Primària de Salut Barcelona Esquerra (CAPSBE), Barcelona, Spain.
  36. Clinical Unit of Rheumatology, School of Medicine, University of l'Aquila, Italy.
  37. Rheumatology Department, Brest University Hospital, Brest, France.
  38. Centre for Experimental Medicine and Rheumatology, Queen Mary University of London, UK.
  39. IRCCS Galeazzi Orthopaedic Institute, Milan and Rheumatology Unit, University of Messina, Italy.
  40. Department of Clinical Science, University of Bergen, and Department of Rheumatology, Haukeland University Hospital, Bergen, Norway.
  41. Department of Internal Medicine, Complexo Hospitalario Universitario de Vigo, Spain.
  42. Division of Rheumatology, Allergy and Immunology, New York University Winthrop Hospital and NYU Langone Health, New York, NY, USA.
  43. Rheumatology Department, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt.
  44. Department of Internal Medicine, Hospital Rey Juan Carlos de Móstoles, Madrid, Spain.
  45. Department of Internal Medicine, Hospital Joan XXIII, Tarragona, Spain.
  46. Department of Rheumatology, Teaching Hospital and University of Montpellier, France.
  47. Marmara University, School of Medicine, Istanbul, Turkey.
  48. Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain.
  49. Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona, Spain.
  50. German Hospital, Buenos Aires, Argentina.
  51. Department of Rheumatology, Hospital 12 de Octubre, Madrid, Spain.
  52. Department of Rheumatology, Hospital de Clínicas, San Lorenzo, Paraguay.
  53. Departamento de Reumatología del Seguro Social Universitario y consultorio privado de Reumatología, Sucre, Bolivia.
  54. Internal Medicine, Hospital Maciel, and Universidad de la República (UdelaR), Montevideo, Uruguay.
  55. Department of Internal Medicine, Hospital 12 de Octubre, Madrid, Spain.
  56. Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Hungary.
  57. Rheumatology Unit, University of Pisa, Italy.
  58. Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Italy.
  59. Department of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, The Netherlands.
  60. Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, México City, Mexico.
  61. Department of Clinical Immunology and Rheumatology, Christian Medical College and Hospital, Vellore, India.
  62. Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
  63. Clinic of Rheumatology, Department of Medical and Biological Sciences, University Hospital "Santa Maria della Misericordia", Udine, Italy.
  64. Department of Rheumatology and Internal Medicine, Wroclaw Medical Hospital, Wroclaw, Poland.
  65. Rheumatology Unit, Department of Medicine, University of Perugia, Italy.
  66. Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, South Korea.
  67. Department of Medicine, Solna, Division of Experimental Rheumatology, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden.
  68. Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, the Netherlands.
  69. Centre 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.
  70. Department of Autoimmune Diseases, ICMiD, University of Barcelona, Hospital Clínic, Barcelona, Spain. mramos@clinic.cat
  71. Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA- Sanitas, Barcelona, Spain.

Sjögren Big Data Consortium

CER14934
2021 Vol.39, N°6 ,Suppl.133
PI 0166, PF 0174
Diagnosis

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PMID: 34919044 [PubMed]

Received: 28/06/2021
Accepted : 06/09/2021
In Press: 16/12/2021
Published: 16/12/2021

Abstract

OBJECTIVES:
To analyse how the main components of the disease phenotype (sicca symptoms, diagnostic tests, immunological markers and systemic disease) can be driven by the age at diagnosis of primary Sjögren’s syndrome (pSS).
METHODS:
By January 2021, the participant centres had included 12,753 patients from 25 countries that fulfilled the 2002/2016 classification criteria for pSS. The age at diagnosis was defined as the time when the attending physician confirmed fulfilment of the criteria. Patients were clustered according to age at diagnosis. 50 clusters with more than 100 observations (from 27 to 76 years) were used to study the influence of the age at diagnosis in the disease expression.
RESULTS:
There was a consistent increase in the frequency of oral dryness according to the age at diagnosis, with a frequency of <90% in patients diagnosed at the youngest ages and >95% in those diagnosed at the oldest ages. The smooth curves that best fitted a linear model were the frequency of dry mouth (adjusted R2 0.87) and the frequency of abnormal oral tests (adjusted R2 0.72). Therefore, for each 1-year increase in the age at diagnosis, the frequency of dry mouth increased by 0.13%, and the frequency of abnormal oral diagnostic tests by 0.11%. There was a consistent year-by-year decrease in the frequency of all autoantibodies and immunological markers except for cryoglobulins. According to the linear models, for each 1-year increase in the age at diagnosis, the frequency of a positive result decreased by 0.57% (for anti-Ro antibodies), 0.47% (for RF) and 0.42% (for anti-La antibodies). The ESSDAI domains which showed a more consistent decrease were glandular and lymph node involvement (for each 1-year increase in the age at diagnosis, the frequency of activity decreased by 0.18%), and constitutional, cutaneous, and haematological involvements (the frequency decreased by 0.09% for each 1-year increase). In contrast, other domains showed an ascending pattern, especially pulmonary involvement (for each 1-year increase in the age at diagnosis, the frequency of activity increased by 0.22%), and peripheral nerve involvement (the frequency increased by 0.09% for each 1-year increase).
CONCLUSIONS:
The influence of the age at diagnosis on the key phenotypic features of pSS is strong, and should be considered critical not only for designing a personalised diagnostic approach, but also to be carefully considered when analysing the results of diagnostic tests and immunological parameters, and when internal organ involvement is suspected at diagnosis.

DOI: https://doi.org/10.55563/clinexprheumatol/egnd1i

Rheumatology Article