Primary Sjögren's syndrome

EBM Guidelines
Sep 3, 2018 • Latest change Dec 18, 2018
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).
  • 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 strongly 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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H04.10, H19.3*M35.0, Internal medicine, M35.0, Ophthalmology, Rheumatology, SSA-antibody, SSB-antibody, Salivary Glands, Schirmer's test, Sjogren, Sjogren syndrome, Sjogren's Syndrome, Sjögren, Sjögren syndrome, Sjögren’s syndrome, Xerophthalmia, Xerostomia, autoimmune exocrinopathy, dry eye disease, dry eye syndrome, keratoconjunctivitis sicca, sicca complex, sicca symptoms, xerophthalmia