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4A43.1 Mikulicz disease

International Classification of Diseases for Mortality and Morbidity Statistics, 11th Revision, v2024-01

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.

synonyms

• Mikulicz disease

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