1 Introduction Sjögren’s syndrome (SS) is a systemic, chronic, autoimmune, inflammatory condition involving the exocrine glands. First defined as “autoimmune epithelitis” by Moutsopoulos, SS is characterized by the presence of lymphocyte infiltrates in glandular tissue that might lead, over time,…
1 Introduction It is well established that environmental factors in concert with an appropriate genetic background play a fundamental role in triggering Sjögren’s syndrome (SS) leading to chronic inflammation of the target organs through different mechanisms, among which the molecular…
Acknowledgments In the past year Professor Simon Bowman has consulted for Eli Lilly, Takeda Pharmaceuticals, UCB, Novartis, Glenmark, Celgene, Medimmune, GSK, and Ono, and is in receipt of an Arthritis Research UK grant to which Roche Pharmaceuticals is contributing rituximab…
1 Spontaneous Models Most of the information from animal models of (Sjögren’s syndrome) SS derives from studies of inbred mouse strains or mice carrying specific genetic defects that spontaneously develop autoimmune sialadenitis. These included New Zealand black (NZB), NZB/New Zealand…
1 Introduction Sjögren’s syndrome (SS) is an autoimmune systemic disease associated with B-cell lymphoproliferation and an increased risk of B-cell non-Hodgkin lymphoma (NHL) development compared with the general population. NHLs are a highly heterogeneous group of malignancies that originate in…
Primary Sjögren’s syndrome (pSS) is an autoimmune disease affecting predominantly the salivary glands. Most of the classification criteria have considered involvement of salivary glands as a major sign of the disease. This involvement can be assessed by imaging procedures such…
1 General Concepts on Classification Criteria Sjögren’s syndrome (SS) is a systemic autoimmune disease (SAD) that primarily affects the exocrine glands, predominantly the salivary and lachrymal glands, and leads to their functional impairment with consequent persistent dryness of the eye…
1 Introduction Sjögren’s syndrome (SS) is a chronic autoimmune exocrinopathy affecting predominantly middle-aged women and displaying lymphocytic infiltration and secretory dysfunction of the exocrine glands (primarily the salivary and lacrimal glands, resulting in oral and ocular dryness). Depending on the…
1 Introduction The clear identification of primary Sjögren’s syndrome (pSS) as a systemic disease occurred only in the 1980s and 1990s. Indeed, in the early 1970s, the only characterized systemic manifestations were bronchitis sicca, interstitial nephritis, and the risk of…
1 Introduction Sjögren’s syndrome (SS) is a complex disorder characterized by both organ-specific and systemic manifestations, potentially expanding to lymphoproliferative complications. In addition to occurring as a primary or secondary disease, SS can occur in association with organ-specific autoimmune diseases,…