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Clinical aspects

 

Systemic manifestations of primary Sjögren's syndrome out of the ESSDAI classification: prevalence and clinical relevance in a large international, multi-ethnic cohort of patients

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

 

  1. Inst. De Investigaciones En Ciencias De La Salud (INICSA), Universidad Nacional de Córdoba (UNC), Consejo Nacional de Investigaciones Científicas y Técnicas (CONICET), Córdoba, and Inst. Universitario de Ciencias Biomédicas de Córdoba (IUCBC), Argentina.
  2. Department of Statistics, Faculty of Science and Letters, Mimar Sinan Fine Arts University, Istanbul, Turkey.
  3. Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, USA.
  4. Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Hungary.
  5. Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.
  6. Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Italy.
  7. Department of Clinical Immunology & Rheumatology, Christian Medical College & Hospital, Vellore, India.
  8. Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Subirán, México City, Mexico.
  9. Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
  10. Department of Rheumatology, University Medical Centre, Ljubljana, Slovenia.
  11. Department of Medicine, Solna, Division of Experimental Rheumatology, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden.
  12. Rheumatology Unit, Department of Medicine, University of Perugia, Italy.
  13. Department of Rheumatology and Internal Medicine, Wroclaw Medical Hospital, Wroclaw, Poland
  14. Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain.
  15. Department of Rheumatology, The Queen Elizabeth Hospital and University of Adelaide, South Australia, Australia.
  16. Rheumatology Division, Hospital das Clinicas, Faculdade de Medicina da Universidade de Sao Paulo (HCFMUSP), Sao Paulo, Brazil.
  17. Department of Medicine, Federal University of Espírito Santo, Vitória, Brazil.
  18. Rheumatology, Department of Medical Sciences, Uppsala University, Uppsala, Sweden.
  19. Department of Rheumatology and Clinical Immunology, University Medical Center Utrecht, Utrecht, The Netherlands.
  20. Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
  21. Otorhinolaryngology, Head and Neck Surgery, Technical University Munich, Munich, Germany.
  22. Clinical Unit of Rheumatology, University of l'Aquila, School of Medicine, L'Aquila, Italy.
  23. Federal University of São Paulo, Brazil.
  24. Rheumatology Department, Brest University Hospital, France.
  25. IRCCS Galeazzi Orthopedic Institute, Milan and Rheumatology Unit, University of Messina, Italy.
  26. Rheumatology Department, Kasr Al Ainy School of Medicine, Cairo University, Egypt.
  27. Internal Medicine, Hospital Maciel, and Universidad de la República (UdelaR), Montevideo, Uruguay.
  28. Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Hungary.
  29. Arthritis and Clinical Immunology Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, USA.
  30. Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Italy.
  31. Department of Clinical Immunology & Rheumatology, Christian Medical College & Hospital, Vellore, India.
  32. Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
  33. Rheumatology Unit, Department of Medicine, University of Perugia, Perugia, Italy.
  34. Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.
  35. Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Subirán, México City, Mexico.
  36. Department of Medicine, Solna, Division of Experimental Rheumatology, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden.
  37. Centre for Immunology of Viral Infections and Autoimmune Diseases, Université Paris-Sud, INSERM UMR1184, Assistance Publique - Hôpitaux de Paris, Hôpitaux Universitaires Paris-Sud, France.
  38. Sjögren’s Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Department of Autoimmune Diseases, ICMiD, University of Barcelona, Hospital Clínic, Spain. mramos@clinic.cat
  39. Sjögren’s Syndrome Res. Group (AGAUR), Lab. of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Dept. of Autoimmune Diseases, ICMiD, Univ. of Barcelona, Hospital Clínic, and Autoimmune Diseases Unit, Dept. of Medicine, Hosp. CIMA-Sanitas, Barcelona, Spain.

on behalf of the Sjögren Big Data Consortium

CER12343
2019 Vol.37, N°3 ,Suppl.118
PI 0097, PF 0106
Clinical aspects

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PMID: 31464664 [PubMed]

Received: 15/04/2019
Accepted : 01/07/2019
In Press: 28/08/2019
Published: 28/08/2019

Abstract

OBJECTIVES:
To analyse the frequency and characterise the systemic presentation of primary Sjögren’s syndrome (SS) out of the ESSDAI classification in a large international, multi-ethnic cohort of patients.
METHODS:
The Big Data Sjögren Project Consortium is an international, multicentre registry based on world-wide data-sharing and cooperative merging of pre-existing clinical SS databases from leading centres in clinical research in SS from the five continents. A list of 26 organ-by-organ systemic features not currently included in the ESSDAI classification was defined according to previous studies; these features were retrospectively recorded.
RESULTS:
Information about non-ESSDAI features was available in 6331 patients [5,917 female, mean age at diagnosis 52 years, mainly White (86.3%)]. A total of 1641 (26%) patients had at least one of the ESSDAI systemic features. Cardiovascular manifestations were the most frequent organ-specific group of non-ESSDAI features reported in our patients (17% of the total cohort), with Raynaud’s phenomenon being reported in 15%. Patients with systemic disease due to non-ESSDAI features had a lower frequency of dry mouth (90.7% vs. 94.1%, p<0.001) and positive minor salivary gland biopsy (86.7% vs. 89%, p=0.033), a higher frequency of anti-Ro/SSA (74.7% vs. 68.7%, p<0.001), anti-La/SSB antibodies (44.5% vs. 40.4%, p=0.004), ANA (82.7% vs. 79.5%, p=0.006), low C3 levels (17.4% vs. 9.7%, p<0.001), low C4 levels (14.4% vs. 9.6%, p<0.001), and positive serum cryoglobulins (8.6% vs. 5.5%, p=0.001). Systemic activity measured by the ESSDAI, clinESSDAI and DAS was higher in patients with systemic disease out of the ESSDAI in comparison with those without these features (p<0.001 for all comparisons).
CONCLUSIONS:
More than a quarter of patients with primary SS may have systemic manifestations not currently included in the ESSDAI classification, with a wide variety of cardiovascular, digestive, pulmonary, neurological, ocular, ENT (ear, nose, and throat), cutaneous and urological features that increase the scope of the systemic phenotype of the disease. However, the individual frequency of each of these non-ESSDAI features was very low, except for Raynaud’s phenomenon.

Rheumatology Article