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Sjögren’s Syndrome

  • Anna Rapti
  • Nikolaos Marketos
  • Clio P. MavraganiEmail author
Chapter

Abstract

Sjögren’s syndrome (SS) is a chronic autoimmune disease of slow progression, with a prevalence of approximately 0.5% in the general population. It mainly affects the exocrine glands of middle-aged women, with the ratio of female to male patients being 9:1. Functional epithelium is gradually affected by lymphocytic infiltrates, resulting in decreased exocrine secretions, the so-called exocrinopathy. As a result, oral, ocular, upper respiratory and vaginal dryness are common complaints. Extraglandular features may also occur. These include nonspecific manifestations (such as Raynaud’s phenomenon, nonerosive polyarthritis, and fatigue), periepithelial infiltrative processes (including interstitial nephritis, liver involvement, and bronchiolitis), and immunocomplex-associated complications (manifested as skin vasculitis, peripheral neuropathy, and glomerulonephritis). The latter are characterized by increased mortality rate due to heightened associated risk for lymphoma development. In approximately two thirds of patients, antibodies against ribonucleoproteins are present. SS is often associated with other organ-specific (autoimmune thyroiditis, primary biliary cirrhosis) and systemic autoimmune disorders (rheumatoid arthritis, lupus, scleroderma). New classification criteria of 2016 include patients that have sicca and/or systemic complaints, with objective ocular findings, unstimulated salivary flow rate, minor salivary gland biopsy findings, and anti-Ro/SSA antibodies being principal components. Treatment modalities focus on symptomatic relief for mild cases and immunomodulation for severe extraglandular manifestations or lymphoma.

Keywords

B-cell activation IFN signaling pathway NF-κB Autoimmune epithelitis Exocrinopathy BAFF Focus score MALT lymphoma 

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Copyright information

© Springer Nature Switzerland AG 2020

Authors and Affiliations

  • Anna Rapti
    • 1
  • Nikolaos Marketos
    • 1
  • Clio P. Mavragani
    • 1
    Email author
  1. 1.Department of Physiology, School of MedicineNational and Kapodistrian University of AthensAthensGreece

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