The value of animal models to study immunopathology of primary human Sjögren's syndrome symptoms

Abstract

Sjögren's syndrome (SjS) is a complex chronic autoimmune disease of multifactorial etiology that results in eventual loss of secretory function in the exocrine glands. The challenges towards finding a therapeutic prevention or treatment for SjS are due primarily to a lack of understanding in the pathophysiological and clinical progression of the disease. In order to circumnavigate this problem, there is a need for appropriate animal models that resemble the major phenotypes of human SjS and deliver a clear underlying biological or molecular mechanism capable of defining various aspects for the disease. Here, we present an overview of SjS mouse models that are providing insight into the autoimmune process of SjS and advance our focus on potential diagnostic and therapeutic targets.

Keywords::

• autoimmunity
• experimentally induced
• Sjögren's syndrome
• mouse models
• spontaneous
• transgenic

Financial & competing interests disclosure

This study was supported in part by PHS grants R00 DE018958 grant (CQ Nguyen) from the NIDCR and funds from the Center for Orphan Autoimmune Disorders. The authors have no other relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript apart from those disclosed.

No writing assistance was utilized in the production of this manuscript.

Key issues

• Treatment and/or prevention of Sjögren's syndrome (SjS) are currently impeded by a lack of understanding pre-disease events and subsequent disease progression.

• While each mouse model of SjS mimics particular disease symptoms or phenotypes, the characteristics vary across models. To date, there is no single mouse model that comprehensively replicates the full breadth of human SjS.

• The combination of methods for development of mouse model (i.e., spontaneous combined with immunization) may mimic disease more thoroughly demonstrating a possible role for both genetic and environmental disease induction.

• Future research should be directed toward continued development of diagnostic and therapeutic strategies and should include a more comprehensive mouse model perhaps designed and built based on ongoing genome-wide association studies.