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Mikulicz syndrome

Mikulicz syndrome was considered a form of Sjögren syndrome (type 1), however, more recently it is considered to be on the IgG4-related disease spectrum. 

It may also be referred to by numerous other eponymous names as well as the catchy: sicca syndrome without a connective tissue disorder 3.

 Clinically, it manifests as:

  • painless bilateral symmetric swelling of the lacrimal and salivary glands
  • xerostomia
  • xerophthalmia

It is a non-specific inflammatory enlargement of at least two or more of the salivary and lacrimal glands with xerostomia

  • IgG4 titers (80% sensitivity)
  • ANA
  • diffuse symmetrical​ enlargement of both parotid glands
  • involvement of other salivary glands
  • bilateral lacrimal gland involvement
  • non-specific lymph node involvement can also occur
  • Low T2 signal intensity (due to high cellularity and fibrosis)
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Article information

rID: 9129
System: Head & Neck
Section: Syndromes
Synonyms or Alternate Spellings:
  • Sicca syndrome without a connective tissue disorder
  • Mikulicz disease
  • Sjogren type 1
  • Dacryosialoadenopathia
  • Dacryosialoadenopathy
  • Mikulicz-Radecki syndrome
  • Mikulicz-Sjogren syndrome
  • von Mikulicz syndrome

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Cases and figures

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