Sjögren’s syndrome is a chronic autoimmune disorder in which the body’s immune system mistakenly attacks and destroys its own moisture-producing glands, especially the salivary and the tear glands. As a result, people with Sjögren’s syndrome typically first experience dry eyes and dry mouth; then there can be dryness in areas like the nose, throat, and skin. Dryness of the eyes is medically called xerophthalmia, and dryness of mouth is called xerostomia.

Primary Sjögren’s is defined as the presence of dryness symptoms in the absence of another connective disease. Secondary Sjögren’s is the term used to define the disorder when it occurs in conjunction with another connective tissue disease. Sjögren’s syndrome usually progresses gradually, but if left untreated it can result in more complex disorders affecting organs like the intestinal tract, kidneys, liver, pancreas, blood vessels, lungs, brain, and joints.

Causes

The exact cause of Sjögren’s syndrome is unknown. It is understood as an autoimmune disease that occurs when white blood cells invade the moisture glands responsible for fluid and mucus production. The result is decreased saliva (dry mouth) decreased tear production (dry eyes) and dryness of other mucus membranes.

Scientists believe that Sjögren’s syndrome is most likely predisposed in Caucasian, Japanese, African American, and Chinese populations, and is caused by the presence of a particular gene. Interestingly, the gene in Caucasians is different from the predisposed gene in Japanese, African American, and Chinese people. Also, the mere presence of the gene does not cause Sjögren’s syndrome; there must also be a trigger that activates the immune system and that trigger can be either a bacterial or viral infection.

For example, a person with the vulnerable gene contracts a viral infection. The virus stimulates the immune system but the gene interferes with the signal and instead of fighting the viral infection, the body sends fighter cells, or lymphocytes, to the glands of the eyes and mouth.

Risk Factors

Between one and four million people in the United States have Sjögren’s syndrome. As discussed in the previous section, a genetic predisposition combined with an environmental trigger is usually present at the onset of Sjögren’s syndrome. The following are the most common risk factors for Sjögren’s syndrome:
 

• Females – 9 out of 10 people with Sjögren’s syndrome are women

 

• Age – the syndrome usually presents in people older than 40

 

• Existing conditions such as rheumatoid arthritis or connective tissue diseases like lupus or scleroderma – these people are more susceptible

 

• A family history of the disease – Sjögren’s syndrome sometimes runs in families

Symptoms of Sjögren’s syndrome vary according to the severity of the disease. The hallmark symptoms of Sjögren’s syndrome include:
 

• Dry mouth for at least three months, including but not limited to difficulty speaking, swallowing, or tasting; difficulty eating dry foods; and, sometimes, cavities and mouth infections

 

• Dry eyes for at least three months, usually with a grainy and itchy feeling in the eyes accompanied by increased sensitivity to bright light and, sometimes, blurry vision

 
Secondary symptoms include:
 

• Feeling extreme fatigue, usually accompanied by body ache

 

• Dryness in other parts of the body such as the nose, throat, vagina, and skin (sometimes presenting with rashes on the skin)

 

• Multiple sites of joint and muscle pain

 

• Numbness or tingling in the extremities

 
In very serious cases, dryness caused by Sjögren’s syndrome may also cause damage to the lung tissue and may result in a persistent, long-lasting cough. The nervous system, kidneys, thyroid, and nerves may also be damaged.

Diagnosis of Sjögren’s syndrome is difficult because the signs and symptoms of the disease vary from person to person. Therefore, a variety of tests are performed in order to rule out other conditions and to confirm Sjögren’s syndrome.

First, a blood test is performed to check the following:
 

• Number of blood cells

 

• Level of glucose

 

• Presence of antibodies specific to Sjögren’s syndrome

 

• Additional clues for any other inflammatory conditions

 

• Any other indicators for potential liver and kidney problems

 
The following two eye tests are also employed by an ophthalmologist, a doctor specializing in eye disorders, in order to arrive at a diagnosis:

• Shirmer test – A small piece of filter paper is placed under the lower eyelid to check the amount of tear produced and measure the dryness of eyes.

 

• Slit-lamp test – Using a magnifying instrument to carefully observe the eyes and check for the severity of dryness and inflammation, the ophthalmologist places a drop of liquid containing dye into the eyes to check for any damage in the cornea.

The salivary glands may also be examined using the following tests:
 

• Sialogram – After a special dye is injected into the parotid gland, located behind the jaw and in front of the ears, then a sialogram (an X-ray of the salivary gland) is taken to check the level of saliva production.

 

• Salivary scintigraphy – Radioactive isotopes are injected and then tracked to measure the level of salivary gland function.

 

• Spit test – The patient spits into a measuring jar every minute, for 15 minutes. Then, the amount of saliva is measured to determine the severity of mouth dryness.

 

• Biopsy – A small sample of the tissue is taken from the salivary glands around the face or under the inner lips testing for the presence of white blood cells to confirm Sjögren’s syndrome

 
A urine test may also be performed to check for any damage to the kidneys. Because Sjögren’s syndrome can cause inflammation of the lung, an X-ray of the chest may be necessary to check the lungs and further pinpoint the diagnosis.

Unfortunately, there is no cure for Sjögren’s syndrome. Treatment for Sjögren’s syndrome is not specific; it depends on the symptoms and the affected organs.

For eye dryness, the doctor may prescribe eye drops. For mouth dryness, the doctor may prescribe some saliva substitutes. The patient can also try chewing sugarless gum and drinking lots of water to prevent mouth dryness. In a few cases, the doctor may prescribe medicine like pilocarpine and cevimeline, both medications that increase saliva production and sometimes, but rarely, improve tear production as well.

Antifungal medicines are employed to treat a yeast infection in the mouth. If the patient complains of arthritis and/or joint pain, then doctor may suggest a non-steroid anti-inflammatory drug, like ibuprofen, to reduce the swelling and pain.

Corticosteroids are given to patients to suppress inflammation that threatens the health of lungs, kidneys, blood vessels, and the nervous system. To soothe dry skin, unscented skin lotions are recommended to moisturize the skin. Saline nasal spray and vaginal lubricants are recommended for patients suffering from dryness. Immunosuppressive drugs like hydroxychloroquine, methotrexate, and cyclophosphamide are prescribed to suppress the activity of immune system. Other medicines may also be prescribed to address specific organs affected by Sjögren’s syndrome.

Outlook
Dryness of eyes and mouth can be an incredibly uncomfortable issue. Though there is no cure for Sjögren’s syndrome, patients with the syndrome, with their physician’s assistance are able to cope and take extra care in keeping their eyes, mouth, and other affected areas hydrated and functional.