Abstract
Primary Sjogren Syndrome (pSS) is a complex, multifactorial rheumatic disease that mainly targets salivary and lacrimal glands, inducing epithelitis. The cause behind the autoimmunity outbreak in pSS is still elusive; however, it seems related to an aberrant reaction to exogenous triggers such as viruses, combined with individual genetic pre-disposition. For a long time, autoantibodies were considered as the hallmarks of this disease; however, more recently the complex interplay between innate and adaptive immunity as well as the consequent inflammatory process have emerged as the main mechanisms of pSS pathogenesis. The present review will focus on innate cells and on the principal mechanisms of inflammation connected. In the first part, an overview of innate cells involved in pSS pathogenesis is provided, stressing in particular the role of Innate Lymphoid Cells (ILCs). Subsequently we have highlighted the main inflammatory pathways, including intra- and extra-cellular players. A better knowledge of such processes could determine the detection of new therapeutic targets that are a major need for pSS.
Highlights
Primary Sjogren Syndrome is an autoimmune exocrinopathy, characterized by xerophthalmia end xerostomia, and caused by a chronic inflammation of lacrimal and salivary glands
The present review aims to clarify the role of innate immunity in Primary Sjogren Syndrome (pSS) development, taking into cells, with a specific interest on new subsets such as innate lymphoid cells (ILCs) and the molecular account all the evidence delivered by the most recent literature
Considering the IFN signature, the most characteristic feature in this disease, it seems important to underline its possible role in clinical setting as a biomarker as well as a therapeutic target
Summary
Primary Sjogren Syndrome (pSS) is an autoimmune exocrinopathy, characterized by xerophthalmia end xerostomia, and caused by a chronic inflammation of lacrimal and salivary glands. PSS can display systemic features affecting extraglandular sites such as joints, vessels, lungs, nerves and kidneys [1] This chronic inflammatory disease can lead approximately 5% of patients to a severe hematological complication such as B-cell non-Hodgkin’s lymphoma. This unfavorable event is mainly due to the hyperactivation and the concomitant disruption of adaptive immunity, as well as to the persistent inflammation at the tissue level [2]. The present review aims to clarify the role of innate immunity in pSS development, taking into cells, with a specific interest on new subsets such as innate lymphoid cells (ILCs) and the molecular account all the evidence delivered by the most recent literature. With a specific interest on new subsets such as innate lymphoid cells (ILCs) and the molecular mechanisms activated by their stimulation
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