Pathogenetic mechanisms in the initiation and perpetuation of Sjögren's syndrome

  title={Pathogenetic mechanisms in the initiation and perpetuation of Sj{\"o}gren's syndrome},
  author={Michael Voulgarelis and Athanasios G. Tzioufas},
  journal={Nature Reviews Rheumatology},
Sjögren's syndrome (SS), a chronic autoimmune disorder, particularly compromises the function of exocrine glands. The involvement of these glands is characterized by focal, mononuclear cell infiltrates that surround the ducts and replace the secretory units. The pathogenetic mechanisms of this autoimmune exocrinopathy have not been fully elucidated. Immunologically-activated or apoptotic glandular epithelial cells that expose autoantigens in genetically predisposed individuals might drive… 
Pathogenetic Mechanisms Implicated in Sjögren’s Syndrome Lymphomagenesis: A Review of the Literature
Sjögren’s Syndrome is a chronic autoimmune disorder characterized by focal mononuclear cell infiltrates that surround the ducts of the exocrine glands, impairing the function of their secretory units, leading to increased morbidity and mortality rates.
Dysregulation of Adaptive Immunity in Sjögren’s Syndrome
  • Qing Yu
  • Medicine, Biology
    Sjögren's Syndrome and Oral Health
  • 2021
Findings from both human samples and mouse disease models have convincingly demonstrated that T and B lymphocytes, the chief cellular players of adaptive immune system cooperatively induce, amplify and sustain SjS disease pathologies through the effector molecules they produce, including cytokines and autoantibodies.
T Cell-Associated Cytokines in the Pathogenesis of Sjögren's Syndrome.
  • Jun-O Jin, Qing Yu
  • Biology, Medicine
    Journal of clinical & cellular immunology
  • 2013
The recent findings on the expression and functions of cytokines in this disease are summarized, with specific focus on those derived from T cells and/or directly affecting T cell responses.
The Multiple Roles of B Cells in the Pathogenesis of Sjögren’s Syndrome
An overview of recent advances in the identification of pathogenic B cell subsets and Breg cells, as well as new development of B-cell targeted therapies in pSS patients are provided.
B-cell hyperactivity in primary Sjögren's syndrome
New insights in the role of B cells in the pathogenetic process of pSS offer ample targets for successful therapeutical intervention in pSS.
B cell dysregulation in primary Sjögren’s syndrome: A review
Unique Phenotypes and Functions of Follicular Helper T Cells and Regulatory T Cells in Sjögren’s Syndrome
This review of recent publications focused on follicular helper T cells related to antibody production and regulatory T cells to control immune tolerance in the pathogenesis of Sjogren’s syndrome.


The Role of Epithelial Cells in the Pathogenesis of Sjögren’s Syndrome
The parallel analysis of SGEC lines obtained fromSS patients and disease controls has revealed the significantly increased constitutive expression of several molecules in cells derived from SS patients, which strongly suggests the operation of intrinsic activation mechanisms in the epithelia of patients and further supports the active participation of epithelIA in SS pathogenesis.
The role of epithelial cells in the initiation and perpetuation of autoimmune lesions: lessons from Sjögren’s syndrome (autoimmune epithelitis)
A model for SS pathogenesis is proposed that integrates the knowledge accumulated during the last decade and reviews all the aspects of the epithelial activity that lead to the perpetuation of the lesion as well as the probable viral factors for the intrinsic activation.
T Lymphocytes in Sjögren’s Syndrome: Contributors to and Regulators of Pathophysiology
The cellular and molecular context of this immune response may drive proinflammatory (Th1 and Th17) and restrain inhibitory (Treg) pathways and define the contributions of participating T cells may unravel strategies for therapeutic intervention.
Sjögren’s Syndrome or Autoimmune Epithelitis?
  • H. Moutsopoulos
  • Medicine, Biology
    Clinical immunology and immunopathology
  • 1994
Clinical studies suggest that the majority of extraglandular manifestations of SS are due to the attraction of lymphocytes by different epithelial tissues and thus the descriptive term "autoimmune epithelitis" instead of "Sjögren's syndrome" is proposed.
Sjögren's syndrome: autoimmune epithelitis.
Based on the sequential application of the validated European classification criteria for SS, a practical algorithm for diagnosis is presented and progress in the understanding of the broad clinicopathological spectrum of the entity remains largely empirical and symptomatic.
Recent advances in understanding molecular mechanisms in the pathogenesis and antibody profile of Sjögren’s syndrome
The effects of adrenopause in Sjögren’s syndrome and on dehydroepiandrosterone and its intracrine metabolism in target tissues can influence the maintenance and remodeling of exocrine glands and may explain, in part, another important disease symptom—fatigue.
Mortality in Sjögren's syndrome.
The role of several factors involved in mortality of sjögren's syndrome, as well as markers predictive for lymphoma development are discussed.
B cells in Sjögren's syndrome: indications for disturbed selection and differentiation in ectopic lymphoid tissue
Glandular B cells expressing distinct features of IgV light and heavy chain rearrangements, (re)circulating B cells with increased mutations of cμ transcripts in both CD27- and CD27+ memory B-cell subsets, and enhanced frequencies of individual peripheral B cells containing IgV heavy chain transcripts of multiple isotypes indicate disordered selection and incomplete differentiation processes of B cells in the inflamed tissues in pSS.
Role of Dendritic Cells in Sjögren's Syndrome
A review will focus on the possible role of DC in SS, which is characterised by progressive mononuclear cell infiltration of the salivary and lacrimal glands and a decreased glandular secretion, resulting in dryness of the mouth and eyes.
Sjögren's syndrome.
  • P. Venables
  • Medicine
    Best practice & research. Clinical rheumatology
  • 2004