4A43.1ICD-11 MMS 2026-01
Mikulicz disease
Description
Mikulicz disease is a disorder first reported by Johann von Mikulicz in 1892 and characterised by symmetrical swelling of the lachrymal, submandibular, and parotid glands, with massive infiltration of these glands by mononuclear cells. Serum autoantibodies, such as anti-Ro/SS-A, are usually negative and serum IgG4 concentration may be increased. Unlike Sjögren disease, IgG4-Mikulicz disease is characterised by the formation of lymphoid follicles, but shows lower levels of lymphocytic infiltration into salivary ducts, such that their structure remains intact.
Index terms
- Mikulicz disease
Exclusions from above levels
Sibling codes
Foundation entity
Part of: 4A43: Overlap or undifferentiated nonorgan specific systemic autoimmune disease › 04: Diseases of the immune system