Mikulicz syndrome was at one time considered to be a form of Sjögren syndrome (type 1), however, it is now considered to be on the IgG4-related disease spectrum.
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Terminology
Mikulicz syndrome may also be referred to by numerous other eponyms as well as the rather verbose "sicca syndrome without a connective tissue disorder" 3.
Epidemiology
Associations
- IgG4-related diseases
- Sjögren syndrome
- gland involvement with sarcoidosis
- gland involvement with lymphoma
Clinical presentation
Clinically, Mikulicz syndrome manifests as:
- painless bilateral symmetric swelling of the lacrimal and salivary glands
- xerostomia
- xerophthalmia
Pathology
Mikulicz syndrome is a non-specific inflammatory enlargement of at least two or more of the salivary and lacrimal glands with xerostomia.
Markers
- IgG4 titers (80% sensitivity)
- ANA
Radiographic features
CT
- diffuse symmetrical enlargement of both parotid glands
- bilateral enlargement of the submandibular glands 4
- bilateral lacrimal gland involvement
- non-specific lymph node involvement can also occur
MRI
- low T2 signal intensity (due to high cellularity and fibrosis)
History and etymology
Mikulicz syndrome is named after the Polish surgeon Jan Mikulicz-Radecki (1850-1905) who first described this disorder in 1892. This explains why it is sometimes known as Mikulicz-Radecki syndrome 5,6.
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