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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.

ORPHA:449432

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: -

  A summary on this disease is available in Español (2022) Français (2022) Nederlands (2022)

Detailed information

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Disease review articles

ERN produced/endorsed by ERN(s)   FSMR produced/endorsed by FSMR(s)

Additional information

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The documents contained in this web site are presented for information purposes only. The material is in no way intended to replace professional medical care by a qualified specialist and should not be used as a basis for diagnosis or treatment.