Immunopathogenesis of Sjögren's syndrome

Andrea T. Borchers, Stanley M Naguwa, Carl L Keen, M. Eric Gershwin

Research output: Contribution to journalArticle

48 Scopus citations

Abstract

Sjögren's syndrome (SS) is an autoimmune disease characterized by the sicca symptoms of dry eyes and dry mouth. Glandular dysfunction is thought to arise from destruction associated with lymphocytic infiltration. The degree of glandular destruction, however, does not correlate with the severity of sicca symptoms, suggesting that other mechanisms are involved, including abnormalities in parasympathetic neurotransmission. Autoantibodies against the muscarinic acetylcholine receptor have been implicated in this process, but multiple other autoantibodies have been found. Cytokines elaborated in the inflammatory lesions also appear to be involved and dysregulation of apoptosis are also involved in the pathogenesis of SS. A new two-stage model of SS has been proposed. First, there is a lymphocyte-independent phase during which inappropriate apoptosis results in the generation of apoptotic autoantigens which then attract lymphocytes. Subsequently, in the second lymphocyte-dependent phase, an immune attack causes further cell death and salivary dysfunction. Although the disease generally takes a rather stable and benign course, patients with SS have a significant risk of developing B cell lymphoma.

Original languageEnglish (US)
Pages (from-to)89-104
Number of pages16
JournalClinical Reviews in Allergy and Immunology
Volume25
Issue number1
DOIs
StatePublished - Aug 2003

Keywords

  • Apoptosis
  • Autoantigens
  • Autoimmune disease
  • Cytokines
  • Sjögren's syndrome

ASJC Scopus subject areas

  • Immunology and Allergy

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