Title:Mouse Models of Primary Sjogren’s Syndrome
VOLUME: 21 ISSUE: 18
Author(s):Young-Seok Park, Adrienne E. Gauna and Seunghee Cha
Affiliation:Department of Oral and Maxillofacial Diagnostic Sciences, University of Florida College of Dentistry, Gainesville, FL32610, USA.
Keywords:Sjogren's syndrome, mouse models, secretory dysfunction, non-obese diabetic (NOD).
Abstract:Sjogren’s syndrome (SjS) is a chronic autoimmune disorder characterized by immune cell infiltration
and progressive injury to the salivary and lacrimal glands. As a consequence, patients with SjS develop xerostomia
(dry mouth) and keratoconjunctivitis sicca (dry eyes). SjS is the third most common rheumatic autoimmune disorder,
affecting 4 million Americans with over 90% of patients being female. Current diagnostic criteria for SjS frequently
utilize histological examinations of minor salivary glands for immune cell foci, serology for autoantibodies,
and dry eye evaluation by corneal or conjunctival staining. SjS can be classified as primary or secondary SjS,
depending on whether it occurs alone or in association with other systemic rheumatic conditions, respectively.
Clinical manifestations typically become apparent when the disease is relatively advanced in SjS patients, which
poses a challenge for early diagnosis and treatment of SjS. Therefore, SjS mouse models, because of their close resemblance to the human
SjS, have been extremely valuable to identify early disease markers and to investigate underlying biological and immunological dysregulations.
However, it is important to bear in mind that no single mouse model has duplicated all aspects of SjS pathogenesis and clinical
features, mainly due to the multifactorial etiology of SjS that includes numerous susceptibility genes and environmental factors. As
such, various mouse models have been developed in the field to try to recapitulate SjS. In this review, we focus on recent mouse models
of primary SjS xerostomia and describe them under three categories of spontaneous, genetically engineered, and experimentally induced
models. In addition, we discuss future perspectives highlighting pros and cons of utilizing mouse models and current demands for improved
models.
