Beyond the Glands: An in-Depth Perspective of Neurological Manifestations in Sjögren’s Syndrome

Primary Sjögren's Syndrome (pSjS) is an autoimmune disease characterized by sicca (xerophthalmia or xerostomia) symptoms, anti-SS-A (Ro) or anti-SS-B (La) autoantibodies, and lymphocytic infiltrates in the exocrine glands. Disease incidence is estimated to be 0.1-3% of the general population with 0.4-3.1 million individuals in the US with women being nine times more likely to be afflicted with SjS than men. The frequency continues to rise accompanied with the multi-factorial etiology making it a challenging disease to manage and treat. Treatment of this disease remains problematic due to the lack of therapeutic treatments relying on replacement therapies such as artificial saliva and eye lubricants or immunosuppressive agents. To further complicate the management of the disease, there are number of multi-systemic manifestations specifically peripheral neuropathy associated with later stage of disease onset. Increasingly, there is mounting evidence that suggests the involvement of central nervous system. It remains to be determined the underlying cause and effect of the dysregulated immune response and the neuropathy associated with SjS. In this review, we provided an in-depth look at key neurological dysfunctions documented to occur in pSjS. Specifically, we discussed the prevalence, symptomology, and current treatments.