4A43.1

ICD-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

  • 02 – Neoplasms
  • 18 – Complications of pregnancy, childbirth and the puerperium
  • 20 – Developmental anomalies

Sibling codes

  • 4A43.0 – IgG4 related disease
  • 4A43.2 – Sjögren syndrome
  • 4A43.3 – Mixed connective tissue disease
  • 4A43.4 – Diffuse eosinophilic fasciitis
  • 4A43.Y – Other specified overlap non-organ specific systemic autoimmune disease
  • 4A43.Z – Undifferentiated non-organ specific systemic autoimmune disease

Foundation entity

Mikulicz disease – http://id.who.int/icd/entity/2012865457

Part of: 4A43: Overlap or undifferentiated nonorgan specific systemic autoimmune disease › 04: Diseases of the immune system