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IgG4-related submandibular gland disease

Disease definition

A rare IgG4-related disease characterized by a benign tumor-like chronic inflammatory lesion of the submandibular gland. Histologic features are periductal fibrosis, acinar atrophy, obliterative phlebitis, dense lymphoplasmacytic infiltrates rich in IgG4-positive plasma cells, and formation of lymphoid follicles. Lobular architecture is preserved. Patients most commonly present with unilateral, painless swelling of the submandibular gland. Serologic analysis reveals elevated IgG4 levels.


Classification level: Subtype of disorder
  • Synonym(s):
    • Küttner tumor
  • Prevalence: Unknown
  • Inheritance: Not applicable 
  • Age of onset: Adult, Elderly
  • ICD-10: K11.2
  • ICD-11: 4A43.0
  • OMIM: -
  • UMLS: C5679995
  • MeSH: -
  • GARD: -
  • MedDRA: -

Detailed information

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