Mikulicz syndromeK11.81

Author:Prof. Dr. med. Peter Altmeyer

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Last updated on: 29.10.2020

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Synonym(s)

IgG4-related dacryoadenitis and sialadenitis; Mikulicz disease; Mikulicz's disease; of Mikulicz disease

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HistoryThis section has been translated automatically.

by Mikulicz-Radecki, 1892

DefinitionThis section has been translated automatically.

Rare, chronic, IgG4-associated (autoimmune) disease of the lacrimal and salivary glands (see below IgG4-associated diseases), which can be monoorganic, but also accompanying in various general and systemic diseases, e.g. Hodgkin's and non-Hodgkin's lymphomas, leukemias and sarcoidosis; also occasionally in tuberculosis, syphilis, sialosis and hyperthyroidism.

EtiopathogenesisThis section has been translated automatically.

Chronic lymphatic leukemia, syphilis or tuberculosis.

ManifestationThis section has been translated automatically.

The syndrome seems to occur preferentially in the Asian population.

LaboratoryThis section has been translated automatically.

High serum levels of IgG4; no detection of SS-A or SS-B antibody (DD Sjögren's syndrome)

DiagnosisThis section has been translated automatically.

Clinic, Sonography

Differential diagnosisThis section has been translated automatically.

Sjögren's syndrome (detection of SS-A or SS-B antibodies).

TherapyThis section has been translated automatically.

Good effectiveness of glucocorticosteroids. Treatment of a possible underlying disease.

Note(s)This section has been translated automatically.

Probably the first description of an IgG4-associated disease in the salivary gland comes from Mikulicz-Radecki . Later work identified the mononuclear infiltrate in the so-called Mikulicz syndrome (K11.8) as an image characterized by IgG4-positive plasma cells.

LiteratureThis section has been translated automatically.

  1. Awan KJ (1976) Mikulicz disease and Mikulicz syndrome. South Afr Med J 69: 458
  2. Azzopardi YES, Evans DJ (1971) Malignant lymphoma of the parotid associated with Mikulicz disease (benign lymphoepithelial lesion). J Clin Pathol 24: 744.
  3. Kowalczyk J (2003) Jan Mikulicz-Radecki (1850-1905)--father of surgical endoscopy. S Afr Med J 93: 509-510
  4. Moriyama M et al (2013) Clinical characteristics of Mikulicz's disease as an IgG4-related disease. Clin Oral Investig 17:1995-2002.
  5. Penfold CN (1985) Mikulicz syndrome. J Oral Maxillofac Surgery 43: 900
  6. by Mikulicz-Radecki (1892) On a peculiar symmetrical disease of the tear and oral salivary glands. In: Contributions on surgery. Commemorative publication dedicated to Theodor Billroth, Stuttgart,pp. 610-630

  7. Shimizu M et al (2015) Effectiveness of imaging modalities for screening IgG4-related dacryoadenitis and sialadenitis (Mikulicz's disease) and for differentiating it from Sjögren's syndrome (SS), with an emphasis on sonography. Arthritis Res Ther 17: 223.

Authors

Last updated on: 29.10.2020

Author: Prof. Dr. med. Peter Altmeyer