What are the other Names for this Condition? (Also known as/Synonyms)

• Chronic Dacryoadenitis and Sialadenitis

What is IgG4-Related Dacryoadenitis and Sialadenitis? (Definition/Background information)

• IgG4-Related Dacryoadenitis and Sialoadenitis (formerly called Mikulicz disease) is an IgG4-related disease characterized by inflammation of the lacrimal glands (which produce tears), parotid glands, and submandibular glands (two of the major salivary glands). In some cases, it also affects other glands or organs
• The condition is usually painless, mainly causing mouth and eye dryness, and swelling over the affected glands. When other organs are affected, it can be accompanied by complications such as autoimmune pancreatitis, retroperitoneal fibrosis, and tubulointerstitial nephritis
• The underlying cause of IgG4-related disease is still not known
• Treatment involves corticosteroids, which are usually effective. Medicines that suppress the immune system (immunosuppressants) may also be used in cases that do not respond to corticosteroids
• IgG4-Related Dacryoadenitis and Sialoadenitis was previously considered a subtype of Sjögren syndrome, but it is now known to be a distinct condition

(Source: Mikulicz Disease; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Who gets IgG4-Related Dacryoadenitis and Sialadenitis? (Age and Sex Distribution)

• IgG4-Related Dacryoadenitis and Sialoadenitis is a rare disorder. The presentation of symptoms may occur at all ages
• Both males and females may be affected
• Worldwide, individuals of all racial and ethnic groups may be affected

What are the Risk Factors for IgG4-Related Dacryoadenitis and Sialadenitis? (Predisposing Factors)

• Currently, no risk factors have been clearly identified for IgG4-Related Dacryoadenitis and Sialoadenitis

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of IgG4-Related Dacryoadenitis and Sialadenitis? (Etiology)

The exact cause of IgG4-Related Dacryoadenitis and Sialoadenitis is not known at the present time.

What are the Signs and Symptoms of IgG4-Related Dacryoadenitis and Sialadenitis?

The signs and symptoms of IgG4-Related Dacryoadenitis and Sialoadenitis may include:

• Dry mouth
• Dry eyes
• Swelling over affected glands (lacrimal, parotid or sub-mandibular glands)

How is IgG4-Related Dacryoadenitis and Sialadenitis Diagnosed?

IgG4-Related Dacryoadenitis and Sialoadenitis is diagnosed on the basis of the following information:

• Complete physical examination
• Thorough medical history evaluation
• Assessment of signs and symptoms
• Laboratory tests, to check for elevated levels of serum IgG4, with lymphocyte and IgG4 positive plasmacyte infiltration
• Imaging studies
• Biopsy studies, if necessary

(Source: Mikulicz Disease; Genetic and Rare Diseases Information Center (GARD) of National Center for Advancing Translational Sciences (NCATS), USA.)

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of IgG4-Related Dacryoadenitis and Sialadenitis?

The complications of IgG4-Related Dacryoadenitis and Sialoadenitis may include:

• Gum infections
• Acid reflux
• Sore throat
• Tooth decay
• Increased risk of eye infections
• Autoimmune pancreatitis 
• Retroperitoneal fibrosis
• Tubulointerstitial nephritis

Complications may occur with or without treatment, and in some cases, due to treatment also.

How is IgG4-Related Dacryoadenitis and Sialadenitis Treated?

The treatment for IgG4-Related Dacryoadenitis and Sialoadenitis may include:

• Corticosteroids
• Immune-suppressing medications in individuals, who do not respond to corticosteroids

How can IgG4-Related Dacryoadenitis and Sialadenitis be Prevented?

• Currently, the exact cause of IgG4-Related Dacryoadenitis and Sialoadenitis is not known, and therefore, no guidelines or methods are available for its prevention 
• Active research is currently being performed to explore the possibilities for treatment and prevention of disorders such as IgG4-Related Dacryoadenitis and Sialoadenitis
• Regular medical screening at periodic intervals with tests and physical examinations are recommended

What is the Prognosis of IgG4-Related Dacryoadenitis and Sialadenitis? (Outcomes/Resolutions)

• The prognosis of IgG4-Related Dacryoadenitis and Sialoadenitis is dependent upon the severity of the signs and symptoms and associated complications, if any
• Individuals with mild conditions have better prognosis than those with severe symptoms and complications
• Typically, the prognosis may be assessed on a case-by-case basis

Additional and Relevant Useful Information for IgG4-Related Dacryoadenitis and Sialadenitis:

IgG4-Related Dacryoadenitis and Sialoadenitis was known by the following terms earlier:

• Mikulicz disease (former)
• Mikulicz syndrome (former)
• Mikulicz's disease (former)

The following DoveMed website link is a useful resource for additional information:

http://www.dovemed.com/diseases-conditions/rare-disorders/