Expression of BAFF and BAFF receptors in primary Sjögren's syndrome patients with ectopic germinal center-like structures

Clin Exp Med. 2020 Nov;20(4):615-626. doi: 10.1007/s10238-020-00637-0. Epub 2020 Jun 6.

Abstract

B cell-activating factor (BAFF) is an essential cytokine in primary Sjögren's syndrome (pSS) physiopathology. It has been reported that pSS patients develop germinal center-like (GC-like) structures in their minor salivary glands (MSGs). BAFF, BAFF-R, TACI, and BCMA expression was analyzed in MSGs from 29 subjects (nonspecific chronic sialadenitis and focal lymphocytic sialadenitis with the presence [pSS-GC(+)] or absence [pSS-GC(-)] of GC-like structures). Twenty-four percent of patients showed ectopic GC-like structures and a high focus score [p < 0.001 vs pSS-GC(-)]. BAFF serum levels (sBAFF) were high in pSS patients (p = 0.025 vs healthy subjects). However, the pSS-GC(-) group showed higher sBAFF levels than pSS-GC(+) patients. BAFF and BAFF-R glandular expression levels were higher in pSS-GC(+) patients, without significant differences compared to pSS-GC(-) patients. Soluble levels of BAFF correlated with anti-La/SSB antibodies and disease duration. Our results showed that BAFF could contribute to focal lymphocytic infiltration. The role of BAFF-binding receptors in MSGs is proposed as a mechanism for the possible establishment of ectopic GC-like structures and disease progression in some patients. In conclusion, this study supports previous evidence that considers the active BAFF system role in the pathogenesis of pSS and the need for strong biomarkers in this disease.

Keywords: BAFF; BAFF receptors; Germinal center; Minor salivary glands; Primary Sjögren’s syndrome.

MeSH terms

  • Adult
  • Aged
  • B-Cell Activating Factor / blood
  • B-Cell Activating Factor / metabolism*
  • B-Cell Activation Factor Receptor / metabolism*
  • B-Cell Maturation Antigen / metabolism
  • Case-Control Studies
  • Female
  • Germinal Center / pathology
  • Humans
  • Immunophenotyping
  • Male
  • Middle Aged
  • Salivary Glands, Minor / pathology*
  • Salivary Glands, Minor / physiology
  • Severity of Illness Index
  • Sjogren's Syndrome / etiology
  • Sjogren's Syndrome / immunology
  • Sjogren's Syndrome / metabolism*
  • Sjogren's Syndrome / pathology
  • Transmembrane Activator and CAML Interactor Protein / metabolism

Substances

  • B-Cell Activating Factor
  • B-Cell Activation Factor Receptor
  • B-Cell Maturation Antigen
  • TNFRSF13B protein, human
  • TNFRSF13C protein, human
  • TNFRSF17 protein, human
  • TNFSF13B protein, human
  • Transmembrane Activator and CAML Interactor Protein

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