The Coexistence of Sjögren's Syndrome and Primary Biliary Cirrhosis: A Comprehensive Review

Ying Sun, Weici Zhang, Baosen Li, Zhengsheng Zou, Carlo F Selmi, M. Eric Gershwin

Research output: Contribution to journalArticle

30 Scopus citations

Abstract

Organ-specific and systemic autoimmune diseases share numerous features and often coexist in the same patient. Autoimmune cholangitis/primary biliary cirrhosis and Sjogren syndrome represent paradigmatic examples of the common grounds of different autoimmunity phenotypes based on similarities in clinical manifestations and immunopathogenesis. In fact, primary biliary cirrhosis and Sjogren’s syndrome have both been coined as an autoimmune epithelitis in which apoptosis may be in both cases the key element to explain the organ-specific immune-mediated injury against the biliary and exocrine gland epithelia, respectively. Further, growing evidence supports in both diseases the view that B cells, T cytotoxic cells, and T helper cells are involved in chronic inflammation, likely via the altered expression of pro-inflammatory cytokines. The presence of estrogen receptors on the biliary and exocrine gland epithelia has been advocated as a key to the female predominance encountered in primary biliary cirrhosis and Sjogren's syndrome. Sadly, despite available data, therapeutic approaches remain largely unsatisfactory and recent studies with mechanistic approaches (as in the case of B cell depletion with rituximab) have been of partial benefit only. Future studies should focus on new molecular tools (single-cell transcriptomics, microRNA, epigenetics) to provide unique insights into common mechanisms.

Original languageEnglish (US)
Pages (from-to)301-315
Number of pages15
JournalClinical Reviews in Allergy and Immunology
Volume48
Issue number2-3
DOIs
StatePublished - Jun 1 2015

Keywords

  • Autoantibody
  • Cholestasis
  • Epithelitis
  • Sicca syndrome
  • Tolerance breakdown

ASJC Scopus subject areas

  • Immunology and Allergy

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