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Sjögren’s Syndrome

  • Eleftherios Pelechas
  • Evripidis Kaltsonoudis
  • Paraskevi V. Voulgari
  • Alexandros A. Drosos
Chapter

Abstract

Sjögren’s syndrome (SS) is a chronic, slowly progressive autoimmune exocrinopathy characterised by mixed cellular infiltration of exocrine glands, notably the lacrimal glands and salivary glands. Xerostomia and xerophthalmia are prominent features of SS, but every epithelial surface can be affected such as nose, throat and vagina. The term autoimmune epitheliitis has been suggested recently. The combination of dry eyes and dry mouth is often referred to as sicca syndrome. SS can be primary or secondary. Secondary SS can be seen in patients with other autoimmune diseases such as rheumatoid arthritis (RA), systemic lupus erythematosus (SLE), scleroderma (SCL) etc. The first description of SS is credited to the Polish surgeon Johann von Mickulicz who, in 1892, described a 42-year-old man with enlargement of the parotid and lacrimal glands associated with a round-cell infiltrate and acinar atrophy. In 1930, Henrik Sjögren coined the term keratoconjunctivitis sicca (KCS) clarifying that this type of xerophthalmia had no relation to vitamin A deficiency. In literature, SS replaced the term keratoconjunctivitis sicca as a more general term.

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Copyright information

© Springer Nature Switzerland AG 2019

Authors and Affiliations

  • Eleftherios Pelechas
    • 1
  • Evripidis Kaltsonoudis
    • 1
  • Paraskevi V. Voulgari
    • 1
  • Alexandros A. Drosos
    • 1
  1. 1.Rheumatology Clinic - Department of Internal MedicineUniversity of IoanninaIoanninaGreece

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