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Review
. 2022 Mar;95(3):e13145.
doi: 10.1111/sji.13145. Epub 2022 Feb 1.

Disease mechanisms in Sjögren's syndrome: What do we know?

Roland Jonsson  1   2
Affiliations
Free article
Review

Disease mechanisms in Sjögren's syndrome: What do we know?

Roland Jonsson. Scand J Immunol. 2022 Mar.
Free article

Abstract

Why should we explore and study disease mechanisms? This is particularly important when we are dealing with complex pathogenesis without a direct causal agent, for example, syndromes with multiple organ involvements. Sjögren's syndrome is definitely such an entity. Also, there are a number of reasons for such studies such as disclosing the aetiology, to identify biomarkers for diagnosis and assessment of the disease process and monitor response to treatment, to determine targets for treatment, to define critical items in classification criteria, amongst others. Samples available for the study of disease mechanisms in Sjögren's syndrome have included serum (autoantibodies, cytokines), DNA (gene profiling, GWAS), cells (phenotypes/flow cytometry, proportion of cells/CyTOF), tissue (focal inflammation, germinal centres, mass cytometry), and saliva (proteomics, biochemistry, mucosal immunity). An original explanatory concept for the pathogenesis of Sjögren's syndrome proposed a specific and self-perpetuating immune-mediated loss of exocrine tissue as the principal cause of glandular hypofunction. This hypothesis however falls short of accommodating several Sjögren's syndrome-related phenomena and experimental findings. Today, the emergence of advanced bio-analytical platforms has further enabled the identification of central pathogenic processes and potential biomarkers. The purpose of this minor review is to highlight a selection of previous but also recent and novel aspects on the disease mechanisms in Sjögren's syndrome.

Keywords: Sjögren's syndrome; autoantibodies; disease mechanisms; genetics; molecular mimicry; pathogenesis; tertiary lymphoid structures.

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