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Rheumatology (Oxford). 2019 Feb 15; doi: 10.1093/rheumatology/key360. Epub 2019 Feb 15.

Innate immunity and interferons in the pathogenesis of Sjögren's syndrome.

Rheumatology (Oxford, England)

Iris L A Bodewes, Albin Björk, Marjan A Versnel, Marie Wahren-Herlenius

Affiliations

  1. Department of Immunology, Erasmus University Medical Centre, Rotterdam, The Netherlands.
  2. Department of Medicine, Rheumatology Unit, Karolinska Institutet, Karolinska University Hospital, Stockholm, Sweden.

PMID: 30770713 DOI: 10.1093/rheumatology/key360

Abstract

Primary SS (pSS) is a rheumatic disease characterized by an immune-mediated exocrinopathy, resulting in severe dryness of eyes and mouth. Systemic symptoms include fatigue and joint pain and a subset of patients develop more severe disease with multi-organ involvement. Accumulating evidence points to involvement of innate immunity and aberrant activity of the type I IFN system in both the initiation and propagation of this disease. Analysis of the activity of IFN-inducible genes has evidenced that more than half of pSS patients present with a so-called 'type I IFN signature'. In this review, we examine activation of the IFN system in pSS patients and how this may drive autoimmunity through various immune cells. We further discuss the clinical value of assessing IFN activity as a biomarker in pSS patients and review novel therapies targeting IFN signalling and their potential use in pSS.

© The Author(s) 2019. Published by Oxford University Press on behalf of the British Society for Rheumatology. All rights reserved. For permissions, please email: [email protected].

Keywords: BAFF; IFN signature; Ro52; SS; fatigue; innate immunity; lymphoma; pDC; therapeutics; type I IFN

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