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Lymphoepithelial lesions in the salivary glands of primary Sjögren’s syndrome patients: the perfect storm?


1, 2, 3, 4, 5, 6, 7, 8

 

  1. Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, The Netherlands. s.a.pringle@umcg.nl
  2. Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, The Netherlands.
  3. Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, The Netherlands.
  4. Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, The Netherlands.
  5. Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, The Netherlands.
  6. Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, The Netherlands.
  7. Department of Pathology and Medical Biology, University of Groningen, University Medical Centre Groningen, The Netherlands.
  8. Department of Rheumatology and Clinical Immunology, University of Groningen, University Medical Centre Groningen, The Netherlands.

CER15979
2022 Vol.40, N°12
PI 2434, PF 2442
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PMID: 36226606 [PubMed]

Received: 22/06/2022
Accepted : 01/09/2022
In Press: 12/10/2022
Published: 20/12/2022

Abstract

In patients with primary Sjögren’s syndrome (pSS), inflamed salivary gland (SG) tissue may contain lymphoepithelial lesions (LELs). LELs are histopathological phenomena whereby B cells are present in hyperplastic ductal epithelium of the SG. Despite the potential role of LELs in pSS pathogenesis, studies on their formation, detection, and prevalence in benign lesions (not complicated with lymphoma) are scarce. Recent evidence however shows that LELs are present in approximately half of the patients with pSS, both in minor and major SGs. Migration of a small number of B cells into the epithelium appears to be a critical initial step in LEL formation. These intra-epithelial B cells are proliferative, exhibit an innate-like phenotype, and may be linked to MALT lymphoma development. Alongside intra-epithelial B cells, the hyperplastic epithelial partner in LELs also engages in the local immune reaction. Epithelial cells are a source of cytokines and chemokines, with CXCL10 in particular playing a potential role in LEL formation. Importantly, LELs also have a negative impact on the maintenance of SG homeostasis by SG progenitor cell (SGPC) populations, likely due to dysregulation of SGPC lineage commitment or induction of plasticity. In conclusion, LEL formation mirrors a perfect storm of B and epithelial cell interaction culminating in increased risk of B cell derailment and SGPC dysregulation in pSS patients. We therefore argue that attenuation of LEL formation is an important treatment goal to preserve SG function and prevent B cell derailment in pSS.

DOI: https://doi.org/10.55563/clinexprheumatol/06an99

Rheumatology Article