Mikulicz syndrome , also known as immunoglobulin G4 (IgG4)-related dacryoadenitis and sialadenitis, 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. 

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

Ultrasound

• parotid and submandibular glands: multiple hypoechoic foci with a reticular pattern ⁷

• submandibular glands: nodal pattern ⁷

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.

Differential diagnosis

• Sjögren syndrome: hypoechoic foci, hyperechoic lines and/or spots in major salivary glands - some overlap with Mikulicz syndrome ⁷