Mikulicz syndrome

Mikulicz syndrome is a rare, chronic disorder characterized by the abnormal enlargement of glandular tissue in the body. Named after the Polish-German physician Johannes von Mikulicz-Radecki, who first described the condition in 1892, the syndrome primarily affects the salivary glands and lacrimal glands, leading to symptoms such as dry mouth and dry eyes.

Symptoms

The most common symptoms of Mikulicz syndrome include persistent swelling in the parotid glands, which are the largest of the salivary glands, and the lacrimal glands, which produce tears. This can result in xerostomia (dry mouth) and keratoconjunctivitis sicca (dry eyes). Other symptoms may include fever, joint pain, and weight loss.

Causes

The exact cause of Mikulicz syndrome is unknown. However, it is often associated with other diseases such as Sjögren's syndrome, lupus, rheumatoid arthritis, and certain types of lymphoma. Some researchers believe that it may be an autoimmune disorder, in which the body's immune system mistakenly attacks its own tissues.

Diagnosis

Diagnosis of Mikulicz syndrome is based on the patient's symptoms, medical history, and physical examination. Imaging tests such as computed tomography (CT) scans and magnetic resonance imaging (MRI) may be used to assess the size and structure of the affected glands. A biopsy of the glandular tissue may also be performed to confirm the diagnosis.

Treatment

Treatment for Mikulicz syndrome primarily involves managing the symptoms. This may include the use of artificial tears and saliva substitutes to alleviate dryness, and nonsteroidal anti-inflammatory drugs (NSAIDs) to reduce inflammation and pain. In severe cases, surgery may be required to remove the affected glands.

Prognosis

The prognosis for individuals with Mikulicz syndrome varies depending on the severity of the symptoms and the presence of associated diseases. With appropriate treatment, most individuals can lead a normal life. However, complications such as infections and dental problems can occur due to the lack of saliva and tears.

See also

• Sjögren's syndrome
• IgG4-related disease
• Salivary gland diseases

NIH genetic and rare disease info

• NIH info on Mikulicz syndrome

Mikulicz syndrome is a rare disease.

Mikulicz syndrome Resources
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