What Are the Forms of Sjögren's Syndrome?
Sjögren's syndrome occurs in two basic forms: primary Sjögren's syndrome -- the disease by itself and not associated with any other illness; and secondary Sjögren's syndrome -- disease that develops in the presence of another autoimmune disease such as rheumatoid arthritis, lupus, or vasculitis.
As many as four million people in the U.S. have Sjögren's syndrome. More than 90% of them are women. The disease can affect people of any race or age, although the average age of onset is the late 40s.
How Is Sjögren's Syndrome Diagnosed?
The diagnosis of primary Sjögren's syndrome is based on several factors, including:
- Presence of dry eyes and mouth. An eye doctor can detect dry eyes by measuring tear production or by carefully examining the status of the tear film of the eye.
- Certain laboratory tests also suggest that dry eyes and mouth are caused by autoimmune mechanisms. Examples include the presence of auto-antibodies in the blood, known as anti-SSA or anti-SSB (also called anti-Ro or anti-La).
- Biopsy of the inner lip (performed in some cases to prove the diagnosis of primary Sjögren's syndrome). The biopsy may show inflammation that is damaging salivary glands.
Secondary Sjögren's syndrome is generally diagnosed when someone with an established autoimmune disease, such as rheumatoid arthritis or lupus, develops extreme dryness of the eyes and mouth. This diagnosis only rarely requires a lip biopsy.
Can Other Problems Mimic Sjögren's Syndrome?
Sometimes, the use of certain drugs can cause side effects that mimic the symptoms of Sjögren's syndrome. Medications such as tricyclic antidepressants (like Elavil or Pamelor) and antihistamines like Benadryl, radiation treatments to the head and neck, as well as other autoimmune disorders, can also cause severely dry eyes and mouth, but it’s not Sjögren’s syndrome.
How is Sjögren's Syndrome Treated?
There is no cure for Sjögren's syndrome, but it can be treated and controlled. The goals of treatment are to decrease discomfort and reduce the harmful effects of dryness. The type of treatment prescribed will be tailored to each patient's symptoms and needs. The following are some of the main methods of treatment for Sjögren's syndrome.
- Good oral hygiene. Good mouth care may not prevent a dry mouth, but it helps prevent infection and cavities. Toothpastes and oral gels are available for people with dry mouth symptoms. These products contain low doses of peroxide. (High amounts can cause more severe dryness.) These products also may have antibacterial action to reduce the severity of dental cavities over a long period of time.
- Increasing eye moisture. Dry eyes are mainly treated with the use of artificial tears, and a wide variety of products are available. Artificial tears must be used regularly and more often in dry environmental conditions such as on airplanes, in air-conditioned buildings, and on windy days.
While artificial tears are helpful, they often do not last long enough. Thicker preparations, like gels or ointments, are available that last longer. These often are used at bedtime because they sometimes cause blurry vision. Eye drops containing cyclosporine (Cequa, Restasis) or lifitegrast ophthalmic solution (Xiidra), treat inflammation in the glands around the eyes and may help to increase tear production. While Cequa or Restasis are often used at bedtime because they sometimes causes blurry vision, Xiidra is used twice a day. Tyrvaya is a newly approved nasal spray that can increase tear production.
Punctal plugs. Tiny plugs can be placed in the tear duct by your eye doctor to keep more lubrication on your eyes. It is painless, and usually takes just a couple of minutes in the exam chair. Surgery to slow the disappearance of tears by sealing the tear ducts with cautery is another treatment option for more severe cases when artificial tears are not sufficient.
- Medications. Drugs that tend to deplete body fluids should be avoided. Mild pain-relieving medications including acetaminophen (Tylenol), Aleve or Motrin can reduce muscle or joint pain. Two prescription drugs, Evoxac and Salagen, stimulate saliva production and may relieve the dry mouth symptoms. In some patients, the anti-rheumatic drug Plaquenil has been beneficial in decreasing pain and salivary gland swelling.
For patients with generalized symptoms, particularly when the disease affects internal organs (including the gastrointestinal system, kidneys, brain, or spinal cord), high doses of immunosuppressive drugs may be necessary. These include medicines such as prednisone and, rarely, chemotherapy-type medications such as methotrexate.
- Balance rest and exercise. Guided exercise programs can help patients overcome fatigue, maintain flexibility, and overcome joint and muscle pain.