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Clinical and Experimental Medicine
Published in: Clinical and Experimental Medicine 1/2022

Open Access 01-02-2022 | Xerophthalmia | Review Article

Sjögren’s syndrome: a systemic autoimmune disease

Authors: Simone Negrini, Giacomo Emmi, Monica Greco, Matteo Borro, Federica Sardanelli, Giuseppe Murdaca, Francesco Indiveri, Francesco Puppo

Published in: Clinical and Experimental Medicine | Issue 1/2022

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Abstract

Sjögren’s syndrome is a chronic autoimmune disease characterized by ocular and oral dryness resulting from lacrimal and salivary gland dysfunction. Besides, a variety of systemic manifestations may occur, involving virtually any organ system. As a result, the disease is characterized by pleomorphic clinical manifestations whose characteristics and severity may vary greatly from one patient to another. Sjögren’s syndrome can be defined as primary or secondary, depending on whether it occurs alone or in association with other systemic autoimmune diseases, respectively. The pathogenesis of Sjögren’s syndrome is still elusive, nevertheless, different, not mutually exclusive, models involving genetic and environmental factors have been proposed to explain its development. Anyhow, the emergence of aberrant autoreactive B-lymphocytes, conducting to autoantibody production and immune complex formation, seems to be crucial in the development of the disease. The diagnosis of Sjögren’s syndrome is based on characteristic clinical signs and symptoms, as well as on specific tests including salivary gland histopathology and autoantibodies. Recently, new classification criteria and disease activity scores have been developed primarily for research purposes and they can also be useful tools in everyday clinical practice. Treatment of Sjögren’s syndrome ranges from local and symptomatic therapies aimed to control dryness to systemic medications, including disease-modifying agents and biological drugs. The objective of this review paper is to summarize the recent literature on Sjögren’s syndrome, starting from its pathogenesis to current therapeutic options.
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Metadata
Title
Sjögren’s syndrome: a systemic autoimmune disease
Authors
Simone Negrini
Giacomo Emmi
Monica Greco
Matteo Borro
Federica Sardanelli
Giuseppe Murdaca
Francesco Indiveri
Francesco Puppo
Publication date
01-02-2022
Publisher
Springer International Publishing
Keyword
Xerophthalmia
Published in
Clinical and Experimental Medicine / Issue 1/2022
Print ISSN: 1591-8890
Electronic ISSN: 1591-9528
DOI
https://doi.org/10.1007/s10238-021-00728-6

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