Immune Netw.  2019 Feb;19(1):e7. 10.4110/in.2019.19.e7.

Aged Sanroque Mice Spontaneously Develop Sjögren's Syndrome-like Disease

• Choi SS
• Jang E
• Oh YK
• Jang K
• Cho ML
• Park SH
• Youn J

• KMID: 2438915
• DOI: http://doi.org/10.4110/in.2019.19.e7

Abstract

Sjögren's syndrome (SS) is a chronic inflammatory autoimmune disorder that affects mainly salivary and lacrimal glands, but its cause remains largely unknown. Clinical data indicating that SS occurs in a substantial proportion of patients with lupus points to common pathogenic mechanisms underlying the two diseases. To address this idea, we asked whether SS develops in the lupus-prone mouse strain sanroque (SAN). Owing to hyper-activation of follicular helper T (Tfh) cells, female SAN mice developed lupus-like symptoms at approximately 20 wk of age but there were no signs of SS at that time. However, symptoms typical of SS were evident at approximately 40 wk of age, as judged by reduced saliva flow rate, sialadenitis, and IgG deposits in the salivary glands. Increases in serum titers of SS-related autoantibodies and numbers of autoantibody-secreting cells in cervical lymph nodes (LNs) preceded the pathologic manifestations of SS and were accompanied by expansion of Tfh cells and their downstream effector cells. Thus, our results suggest that chronic dysregulation of Tfh cells in salivary gland-draining LNs is sufficient to drive the development of SS in lupus-prone mice.

Keyword

Sjögren's syndrome; Animal disease models; Systemic lupus erythematosus; Autoimmunity; Follicular helper T cell

MeSH Terms

Animals
Autoantibodies
Autoimmunity
Disease Models, Animal
Female
Humans
Immunoglobulin G
Lacrimal Apparatus
Lupus Erythematosus, Systemic
Lymph Nodes
Mice*
Saliva
Salivary Glands
Sialadenitis
Autoantibodies
Immunoglobulin G