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Primary Sjögren syndrome

Disease definition

A rare systemic autoimmune disease characterized by exocrine gland dysfunction, resulting predominately in keratoconjunctivitis sicca and xerostomia, but also affecting exocrine glands of the skin, as well as respiratory, urogenital, and digestive tract. Extraglandular manifestations include arthritis, interstitial lung disease, renal disease, and peripheral neuropathy. The disease is accompanied by a substantially increased risk to develop B-cell non-Hodgkin lymphoma, especially MALT (mucosa-associated lymphoid tissue) lymphoma.


Classification level: Disorder
  • Synonym(s):
    • Primary Sjögren-Gougerot syndrome
  • Prevalence: 1-5 / 10 000
  • Inheritance: Not applicable 
  • Age of onset: Adult
  • ICD-10: M35.0
  • ICD-11: 4A43.20
  • OMIM: 270150
  • UMLS: C0151449
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information

General public


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