IgG4-related dacryoadenitis and sialadenitis

Orpha code: 79078OMIM code:

Definition

IgG4-related dacryoadenitis and sialoadenitis (Mikulicz disease) is an IgG4-related sclerosing disease (see this term) characterized by persistent, usually painless, bilateral enlargement of the lacrimal, parotid, and submandibular glands associated with elevated levels of serum immunoglobulin (Ig) G4 and with lymphocyte and IgG4-positive plasmacyte infiltration. It predominantly causes mouth and eye dryness but can also affect other organs such as the lungs, liver, and kidneys, and be accompanied by complications such as autoimmune pancreatitis (AIP), retroperitoneal fibrosis, and tubulointerstitial nephritis (see these terms).

Disease data
Classification

Clinical subtype

Synonyms
Chronic dacryoadenitis and sialadenitis
Choroba Mikulicza
Przewlekłe zapalenie gruczołów łzowych i ślinianek
Mikulicz disease
ORPHA code
79078
OMIM code
-
ICD10 code
K11.8
ICD11 code
4A43.0

No additional description.

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