Primary Sjögren's syndrome

EBM Guidelines
Oct 13, 2023 • Latest change May 28, 2025
Heikki Julkunen

Table of contents

Extract

  • Primary or essential Sjögren's syndrome is an autoimmune disease characterized by chronic lymphocytic inflammation of exocrine glands and resulting glandular hypofunction and dryness of mucosa.
  • Its primary symptoms are dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia).
  • Some patients also experience symptoms outside the exocrine glands and general symptoms.
  • Presence of autoantibodies (rheumatoid factor, anti-nuclear antibodies, ENAAb) is typical for the disease. Their absence in patients suffering from dry eyes and dry mouth speaks against the diagnosis.
  • Topical treatment is used for dryness of mucosa. Extraglandular symptoms and complicated disease may be treated with glucocorticoids, hydroxychloroquine and immunosuppressive medication.
  • Secondary Sjögren's syndrome may occur in patients with other autoimmune diseases.

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Search terms

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