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Combined seronegativity in Sjögren’s syndrome


1, 2, 3, 4, 5, 6, 7, 8, 9, 10, 11

 

  1. Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, and Institute for Autoimmune, Systemic and Neurological Diseases, Athens, Greece.
  2. Unit of Medical Technology and Intelligent Information Systems, University of Ioannina, Greece.
  3. Rheumatology Clinic, Department of Internal Medicine, Medical School, University of Ioannina, Greece.
  4. Rheumatology Unit, Department of Clinical and Experimental Medicine, University of Pisa, Italy.
  5. Unit of Medical Technology and Intelligent Information Systems, University of Ioannina, Greece.
  6. Unit of Medical Technology and Intelligent Information Systems, University of Ioannina, and Department of Biomedical Research, Institute of Molecular Biology and Biotechnology, FORTH, Ioannina, Greece.
  7. Department of Physiology, Medical School, National and Kapodistrian University of Athens, Greece.
  8. Department of Nutrition and Clinical Dietetics, Harokopio University of Athens, and Department of Medicine and Clinical Immunology, Euroclinic of Athens, Greece.
  9. Institute for Autoimmune, Systemic and Neurological Diseases, Athens, and Athens Academy of Athens, Chair Medical Sciences/Immunology, Greece.
  10. Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, and Institute for Autoimmune, Systemic and Neurological Diseases, Athens, Greece.
  11. Department of Pathophysiology, School of Medicine, National and Kapodistrian University of Athens, and Institute for Autoimmune, Systemic and Neurological Diseases, Athens, Greece. agoules@med.uoa.gr

CER14929
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PMID: 34665703 [PubMed]

Received: 25/06/2021
Accepted : 06/09/2021
In Press: 04/10/2021

Abstract

OBJECTIVES:
To describe the clinical spectrum of Sjögren’s syndrome (SS) patients with combined seronegativity.
METHODS:
From a multicentre study population of consecutive SS patients fulfilling the 2016 ACR-EULAR classification criteria, patients with triple seronegativity [anti-Ro/SSA(-), anti-La/SSB(-), RF(-) and ANA(+)] and quadruple seronegativity [anti-Ro/SSA(-), anti-La/SSB(-), RF(-) and ANA(-)] were identified retrospectively. Both groups were matched in an 1:1 ratio with 2 distinct control SS groups: i) classic anti-Ro/SSA seropositive patients [SS(+)] and ii) classic anti-Ro/SSA seropositive patients with negative rheumatoid factor [SS(+)/RF(-)] to explore their effect on disease expression. Clinical, laboratory and, histologic features were compared. A comparison between triple and quadruple seronegative SS patients was also performed. REESULTS: One hundred thirty-five SS patients (8.6%) were identified as triple seronegative patients and 72 (4.5%) as quadruple. Triple seronegative patients had lower frequency of peripheral nervous involvement (0% vs. 7.2% p=0.002) compared to SS(+) controls and lower frequency of interstitial renal disease and higher prevalence of dry mouth than SS(+)/RF(-) controls. Quadruple seronegative patients presented less frequently with persistent lymphadenopathy (1.5% vs. 16.9 p=0.004) and lymphoma (0% vs. 9.8% p=0.006) compared to SS(+) controls and with lower prevalence of persistent lymphadenopathy (1.5% vs. 15.3% p=0.008) and higher frequency of dry eyes (98.6% vs. 87.5% p=0.01) and autoimmune thyroiditis (44.1% vs. 17.1% p=0.02) compared to SS(+)/RF(-) SS controls. Study groups comparative analysis revealed that triple seronegative patients had higher frequency of persistent lymphadenopathy and lymphoma, higher focus score and later age of SS diagnosis compared to quadruple seronegative patients.
CONCLUSIONS:
Combined seronegativity accounts for almost 9% of total SS population and is associated with a milder clinical phenotype, partly attributed to the absence of rheumatoid factor.

Rheumatology Article