What is Mikulicz Syndrome?

Mikulicz syndrome is a chronic disease in which the glandular tissue of the head and neck are excessively enlarged, usually bilaterally. Mostly, the salivary (parotid) and lacrimal (tear-duct) glands are affected.

As a consequence of lacrimal gland enlargement, the most common effect is dry eyes. Parotid gland enlargement may also be associated with extreme dryness in the mouth (xerostomia), leading to difficulty in swallowing dry foods, and in some cases, tooth decay.

This condition is very closely related to Sjögren syndrome, and may actually be a different form of the same condition. Sjögren syndrome is also characterized by a dry mouth and dry eyes. Dry eyes can lead to blurry vision or intolerance to bright lighting.

People with Mikulicz syndrome and Sjögren syndrome have an increased risk of developing non-Hodgkin lymphoma. Mikulicz syndrome tends to affect females more than males, and tends to occur in adults over the age of 50.  

What causes Mikulicz syndrome?

In almost all cases of Mikulicz syndrome, there is an underlying cause. These can include, but are not limited to, leukemia, Hodgkin’s disease, AIDs, syphilis, lupus and Sjögren syndrome.

The exact cause of Mikulicz syndrome is still not fully understood; however most scientists now believe it to be a form of Sjögren syndrome. This is presently considered to be autoimmune in nature, leading to the accumulation of lymphocytes in the affected glands of the face, mouth and neck. More specifically, research has linked it to IgG-4 related dysfunction.

Are there any treatments for Mikulicz syndrome?

There is no definitive curative treatment for either Mikulicz or Sjögren syndrome. Most therapies are directed at alleviating the symptoms of dry eyes and mouth. For example, ophthalmic ointment, artificial tears, medicated sprays for the throat, or medications that increase the production of saliva and tears, may be offered. Clinicians may also encourage lifestyle changes, such as abstinence from alcohol, smoking and caffeine, as well as incorporating naturally wet foods into the diet to help moisten the mouth while eating, and so facilitate swallowing.

Careful observation and management of symptoms is often needed, especially if symptoms are severe and persistent. Sometimes the symptoms may improve spontaneously for short periods of time, although it is a chronic disorder and tends to linger lifelong.

Further Reading

• All Mikulicz Syndrome Content

Written by

Osman Shabir

Osman is a Neuroscience PhD Research Student at the University of Sheffield studying the impact of cardiovascular disease and Alzheimer's disease on neurovascular coupling using pre-clinical models and neuroimaging techniques.