IgG4-related dacryoadenitis and sialadenitis
Findings
No curated finding names IgG4-related dacryoadenitis and sialadenitis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.
Definition
IgG4-related dacryoadenitis and sialoadenitis (Mikulicz disease) is an IgG4-related sclerosing disease 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.
Definition from the Mondo Disease Ontology (MONDO:0019191), read 2026-09-29. CC BY 4.0.
Features
33 features
What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.
- Abnormal salivary gland morphologyHPOHP:0010286
- Very frequent (80% to 99% of cases)
- Abnormality of the submandibular glandsHPOHP:0010287
- Very frequent (80% to 99% of cases)
- Enlarged lacrimal glandsHPOHP:0007734
- Very frequent (80% to 99% of cases)
- Increased circulating IgG4 concentrationHPOHP:0032300
- Very frequent (80% to 99% of cases)
- Enlargement of parotid glandHPOHP:0011801
- Frequent (30% to 79% of cases)
- Abnormal optic nerve morphologyHPOHP:0000587
- Occasional (5% to 29% of cases)
Show the remaining 21
- FatigueHPOHP:0012378
- Occasional (5% to 29% of cases)
- FeverHPOHP:0001945
- Occasional (5% to 29% of cases)
- Generalized muscle weaknessHPOHP:0003324
- Occasional (5% to 29% of cases)
- Increased circulating IgA concentrationHPOHP:0003261
- Occasional (5% to 29% of cases)
- Increased circulating IgM concentrationHPOHP:0003496
- Occasional (5% to 29% of cases)
- Keratoconjunctivitis siccaHPOHP:0001097
- Occasional (5% to 29% of cases)
Where it sits
Other names
2 names
Resolves to: IgG4-related dacryoadenitis and sialadenitis
- Also called
- chronic dacryoadenitis and sialadenitisMikulicz disease