What is Sjogren's Syndrome?

Sjogren's syndrome is an autoimmune-mediated systemic inflammatory disorder found in about 0.5-1% of the population.  It affects different parts of the body, but the most severe symptoms are typically associated with the tear glands and salivary glands. The condition is specifically characterized by the presence of a dry mouth and dry eyes (sicca symptoms).

Primary Sjogren's syndrome occurs in people with no underlying cause, whereas the secondary form of the disorder is caused by an underlying rheumatologic disease. Systemic lupus erythematosus and rheumatoid arthritis are the two leading causes of secondary Sjogren's syndrome.

What are the symptoms of Sjogren's syndrome?

Dryness in the eyes causes painful burning sensations. The eyes may also feel gritty. The presence of dryness in the mouth makes it difficult to eat and swallow food. Some individuals may also encounter extreme dryness in the throat, nasal pathways and vagina. Dry skin and swelling of the face and neck gland is also common.

In up to half of all cases the joints, skin, gut, nervous system, and kidneys are also involved by Sjogren’s syndrome.

Complications of Sjogren's syndrome

The complications associated with Sjögren's syndrome are basically related to its effects on the tear and salivary glands. Tears are the cleansing system of the eyes and hence dry eyes put the individual at an increased risk of developing infections. Tears are also involved in the supply of nutrients to the cornea; for this reason, dry eyes can also lead to corneal damage.

Similarly, the lack of saliva is also associated with an increased risk of dental problems such as tooth decay, thrush and gingivitis. Patients may also encounter swelling of the salivary glands. Painful stiff joints, fatigue and numbness have also been reported in some patients.

In primary Sjogren’s syndrome, the risk of B-cell non-Hodgkin’s lymphoma is increased compared to the general population, and often involve the salivary glands. Warning signs of this change must be looked for and these patients carefully followed up for proper referral when necessary.

Vascular thrombotic disorders are more common in pregnant women with Sjogren’s syndrome and this may cause a high complication rate, including miscarriages and premature labor.

What are the risk factors for Sjogren's syndrome?

Sjogren's syndrome can develop at any age; however, the condition is usually found in people older than 40. The syndrome is nine times more common in women. About half of people suffering from rheumatic disease also have Sjogren's syndrome.

How is Sjogren's syndrome diagnosed?

The differential diagnosis of Sjogren's syndrome is crucial to initiate the right treatment strategy. The presence of a plethora of symptoms and its marked resemblance to other diseases makes accurate diagnosis a challenge.

Blood tests for disease-specific antibodies can help in the diagnosis of Sjogren's syndrome. Other tests include the counts of different blood cells and inflammatory mediator concentrations.

Schirmer tear test is used to measure the amount of tears produced by the eyes and to assess eye function.

Sialography and salivary scintigraphy are two imaging tests that may be utilized to determine the function of the salivary glands.

In certain instances, a lip biopsy may be recommended to detect specific inflammatory cells associated with Sjogren's syndrome.

What are the treatment options for Sjogren's syndrome?

For mild cases of Sjogren's syndrome, the use of over-the-counter eye drops (without preservatives)  or gels coupled with increased water intake can help to manage the symptoms of dry eyes and dry mouth respectively. Artificial tears and artificial saliva may also help to relieve the discomfort.

Proper dental care is necessary to prevent caries, and includes the use of fluoridated toothpaste, as well as stimulation of saliva by sugar-free chewing gum or lozenges.

Warm compresses help relieve the dryness, as does stopping the use of contact lenses. Ophthalmologic consultation is typically required.

Severe cases may need pharmacological treatment, and in some cases surgery. Eye drops containing anti-inflammatory agents are often prescribed to combat inflammation. Drugs belonging to the class of cholinergic agonists can help in increasing saliva production. Nonsteroidal anti-inflammatory drugs (NSAIDs) may be used to tackle the arthritis-like symptoms.

Punctal occlusion is a minor surgical operation which may help in treating the dry eyes associated with Sjogren's syndrome. The procedure involves sealing the tear ducts with collagen or silicone plugs.

Self-care habits such as avoiding smoking and good oral hygiene can also help relieve the symptoms to some extent.

Further Reading

Last Updated: Apr 15, 2019


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