Sjögren’s syndrome: a systemic autoimmune disease

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.

Autoimmune Connective Tissue Diseases-Related Pruritus: Proper Diagnosis and Possible Mechanisms

“…SS is an autoimmune disease characterized by dysfunction of salivary and lacrimal glands. It can be defined as primary SS when it occurs alone or secondary SS if systemic autoimmune diseases accompany [ 65 ]. Extra-glandular involvement is common, especially dermatological involvement.…”

“…Further investigations of the possible mechanisms of xerosis and the relationship between xerosis and pruritus in SS are necessary. Peripheral nervous system involvement with sensory neuropathy in SS is common [ 65 , 70 ]. Small-fiber neuropathy has been demonstrated in primary SS and higher neuropathic symptom burden compared to other well-known small-fiber neuropathy etiologies, hereditary transthyretin amyloidosis and idiopathic small-fiber neuropathy [ 71 ].…”

“Listen to Your Immune System When It’s Calling for You”: Monitoring Autoimmune Diseases Using the iShU App

“…Sjögren’s Syndrome is portrayed by the occurrence of periductal lymphocytic infiltrations in salivary and lachrymal glands resulting in oral and ocular dryness [ 68 ]. Typical signs are dry eyes and mouth (i.e., xerophthalmia and xerostomia), dry skin, fatigue, pain, dysphagia, distorted taste sensation (i.e., dysgeusia), burning impression, dental caries, periodontal disease, photosensitivity, redness, itching, hoarseness, nonproductive cough, painful intercourse (i.e., dyspareunia), sleep disorder, anxiety and depressive symptoms, stiffness, pain in muscles (i.e., myalgia), arthralgia, weakness, pallor, cutaneous vasculitis, and paralysis [ 69 ].…”

Umbilical cord mesenchymal stem cells alleviate Sjögren’s syndrome and related pulmonary inflammation through regulating Vγ4+ IL-17+ T cells

“…Primary Sjögren's syndrome (SS) is a typical autoimmune disease characterized by progressive inflammation, tissue damage in salivary glands (SGs) and lacrimal glands (LGs), and reduced production of saliva and tears which may lead to symptoms like dry mouth and dry eye (1)(2)(3). It also impairs some organs, including those of the renal, respiratory, and nervous systems.…”