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.

Terminology

It may also be referred to by numerous other eponyms as well as the rather verbose: sicca syndrome without a connective tissue disorder .

Epidemiology

Associations

• IgG4-related diseases
• Sjögren syndrome
• gland involvement with sarcoidosis
• gland involvement with lymphoma

Clinical presentation

 Clinically, it manifests as:

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

Pathology

It 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
• involvement of other salivary glands
• bilateral lacrimal gland involvement
• non-specific lymph node involvement can also occur

MRI 

• low T2 signal intensity (due to high cellularity and fibrosis)

Siehe auch:

• Sarkoidose
• Warthin-Tumor
• Sjögren-Syndrom
• Sialadenitis
• lymphoepitheliale Zyste der Glandula parotis
• Vergrößerung der Glandula parotis
• salivary
• lymphoepitheliale Zyste des Pankreas

und weiter:

• zystische Läsionen der Glandula parotis