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Clinical aspects

 

Systemic phenotype related to primary Sjögren's syndrome in 279 patients carrying isolated anti-La/SSB antibodies

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

 

  1. Department of Statistics and Operations Research, Universitat Politècnica de Catalunya, Barcelona, Spain.
  2. Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Hungary.
  3. Department of Rheumatology, Skane University Hospital Malmö, Lund University, Sweden.
  4. Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Italy.
  5. Department of Oral and Maxillofacial Surgery, University of Groningen, University Medical Center Groningen, The Netherlands.
  6. Immunology and Rheumatology Department, Instituto Nacional de Ciencias Médicas y Nutrición Salvador Zubirán, México City, Mexico.
  7. Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
  8. Department of Rheumatology, University Medical Centre, Ljubljana, Slovenia.
  9. Department of Rheumatology and Internal Medicine, Wroclaw Medical University, Wroclaw, Poland.
  10. Rheumatology Unit, Department of Medicine, University of Perugia, Italy.
  11. Department of Rheumatology, The Queen Elizabeth Hospital, Discipline of Medicine, University of Adelaide, SA, Australia.
  12. Rheumatology Division, Hospital das Clinicas HCFMUSP, Faculdade de Medicina, Universidade de Sao Paulo, Brazil.
  13. Rheumatology, Department of Medical Sciences, Uppsala University, Sweden.
  14. Department of Immunology and Rheumatology, Division of Advanced Preventive Medical Sciences, Nagasaki University Graduate School of Biomedical Sciences, Nagasaki, Japan.
  15. Federal University of São Paulo, Brazil.
  16. Instituto Modelo de Cardiología Privado SRL, Córdoba, and Instituto Universitario de Ciencias Biomédicas de Córdoba (IUCBC), Córdoba, Argentina.
  17. Division of Rheumatology, Allergy and Immunology, NYU Long Island School of Medicine, Mineola, NY, USA.
  18. Department of Autoimmune Diseases, Complexo Hospitalario Universitario de Vigo, Spain.
  19. Department of Internal Medicine, Hospital Rey Juan Carlos de Móstoles, Madrid, Spain.
  20. Department of Internal Medicine, Hospital Joan XXIII, Tarragona, Spain.
  21. Department of Internal Medicine, Hospital de Cabueñes, Gijón, Spain.
  22. Department of Rheumatology, Hospital 12 de Octubre, Madrid, Spain.
  23. Department of Rheumatology, Hospital de Clínicas, San Lorenzo, Paraguay.
  24. Departamento de Reumatología del Seguro Social Universitario y consultorio privado de Reumatología, Sucre, Bolivia.
  25. Department of Internal Medicine, Hospital 12 de Octubre, Madrid, Spain.
  26. Institute of Cellular Medicine, Newcastle University, and NIHR Biomedical Research Centre, Newcastle Upon Tyne, UK.
  27. Genes and Human Disease Research Program, Oklahoma Medical Research Foundation, Oklahoma City, OK, USA.
  28. Department of Rheumatology, Peking Union Medical College Hospital, Beijing, China.
  29. Department of Rheumatology and Immunology, Anhui Provincial Hospital, Hefei, China.
  30. Rheumatology Unit, University of Pisa, Italy.
  31. 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.
  32. Department of Rheumatology, Strasbourg University Hospital, Université de Strasbourg, CNRS, Strasbourg, France.
  33. Department of Rheumatology and Clinical Immunology, University Medical Centre Utrecht, The Netherlands.
  34. Department of Clinical Immunology & Rheumatology, Christian Medical College & Hospital, Vellore, India.
  35. Clinic of Rheumatology, Department of Medical and Biological Sciences, University Hospital 'Santa Maria della Misericordia', Udine, Italy.
  36. Division of Rheumatology, Department of Internal Medicine, Seoul St. Mary's Hospital, College of Medicine, The Catholic University of Korea, Seoul, South Korea.
  37. Department of Medicine, Solna, Division of Experimental Rheumatology, Karolinska Institutet, and Karolinska University Hospital, Stockholm, Sweden.
  38. Department of Internal Medicine, Hospital Vall d'Hebron, Barcelona, Spain.
  39. Service de Médecine Interne 2, Hôpital Lariboisière, Université Paris VII, Assistance Publique-Hôpitaux de Paris, France.
  40. Division of Rheumatology , Kanazawa University Hospital, Kanazawa, Ishikawa, Japan.
  41. Centre for Rheumatology, Division of Medicine, University College London, UK.
  42. Department of Medicine, Federal University of Espírito Santo, Vitória, Brazil.
  43. Otorhinolaryngology, Head and Neck Surgery, Technical University Munich, Germany.
  44. Clinical Unit of Rheumatology, School of Medicine, University of L’Aquila, Italy.
  45. Rheumatology Department, Brest University, INSERM 1227, Brest, France.
  46. IRCCS Galeazzi Orthopaedic Institute, Milan and Rheumatology Unit, University of Messina, Italy.
  47. Rheumatology Department, Kasr Al Ainy School of Medicine, Cairo University, Cairo, Egypt.
  48. Department of Rheumatology, Teaching Hospital and University of Montpellier, France.
  49. Department of Internal Medicine and Medical Specialties, Rheumatology Clinic, Sapienza University of Rome, Italy.
  50. Department of Internal Medicine, Hacettepe University, Faculty of Medicine, Ankara, Turkey.
  51. Division of Clinical Immunology, Faculty of Medicine, University of Debrecen, Hungary.
  52. Department of Rheumatology, Skane University Hospital Malmö, Lund University, Sweden.
  53. Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, the Netherlands.
  54. 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.cat
  55. Department of Statistics and Operations Research, Universitat Politècnica de Catalunya, Barcelona, and Primary Healthcare Transversal Research Group, Institut d’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), Primary Care Centre Les Corts, Consorci d’Atenció Primària de Salut Barcelona Esquerra (CAPSBE), Barcelona, Spain.
  56. Sjögren Syndrome Research Group (AGAUR), Laboratory of Autoimmune Diseases Josep Font, IDIBAPS-CELLEX, Department of Autoimmune Diseases, ICMiD, University of Barcelona, Hospital Clínic, Barcelona, and Autoimmune Diseases Unit, Department of Medicine, Hospital CIMA-Sanitas, Barcelona, Spain.

for the Sjögren Big Data Consortium

CER13941
2020 Vol.38, N°4 ,Suppl.126
PI 0085, PF 0094
Clinical aspects

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PMID: 33095152 [PubMed]

Received: 17/08/2020
Accepted : 03/09/2020
In Press: 22/10/2020
Published: 22/10/2020

Abstract

OBJECTIVES:
To evaluate the systemic phenotype associated with the presence of isolated anti-La/SSB antibodies in a large international registry of patients with primary Sjögren’s syndrome (pSS) fulfilling the 2002 classification criteria.
METHODS:
The Big Data Sjögren Project Consortium is an international, multicentre registry created in 2014. Baseline clinical information from leading centres on clinical research in SS of the 5 continents was collected. Combination patterns of anti-Ro/SSA-La/SSB antibodies at the time of diagnosis defined the following four immunological phenotypes: double positive (combined Ro/SSA and La/SSB,) isolated anti-Ro/SSA, isolated anti-La/SSB, and immunonegative.
RESULTS:
The cohort included 12,084 patients (11,293 females, mean 52.4 years) with recorded ESSDAI scores available. Among them, 279 (2.3%) had isolated anti-La/SSB antibodies. The mean total ESSDAI score at diagnosis of patients with pSS carrying isolated anti-La/SSB was 6.0, and 80.4% of patients had systemic activity (global ESSDAI score ≥1) at diagnosis. The domains with the highest frequency of active patients were the biological (42.8%), glandular (36.8%) and articular (31.2%) domains. Patients with isolated anti-La/SSB showed a higher frequency of active patients in all ESSDAI domains but two (articular and peripheral nerve) in comparison with immune-negative patients, and even a higher absolute frequency in six clinical ESSDAI domains in comparison with patients with isolated anti-Ro/SSA. In addition, patients with isolated anti-La/SSB showed a higher frequency of active patients in two ESSDAI domains (pulmonary and glandular) with respect to the most active immunological subset (double-positive antibodies). Meanwhile, systemic activity detected in patients with isolated anti-La/SSB was overwhelmingly low. Even in ESSDAI domains where patients with isolated anti-La/SSB had the highest frequencies of systemic activity (lymphadenopathy and muscular), the percentage of patients with moderate or high activity was lower in comparison with the combined Ro/SSA and La/SSB group.
CONCLUSIONS:
Patients carrying isolated La/SSB antibodies represent a very small subset of patients with a systemic SS phenotype characterised by a significant frequency of active patients in most clinical ESSDAI domains but with a relative low frequency of the highest severe organ-specific involvements. Primary SS still remains the best clinical diagnosis for this subset of patients.

Rheumatology Article