Sjögren's syndrome is a chronic disorder that causes damage to the salivary glands, resulting in dry mouth, and the tear glands, resulting in dry eyes. It can also affect other parts of the body including joints, muscles and nerves, organs such as the lungs, kidneys, liver, pancreas, stomach and brain, or glands such as the thyroid gland. Sjögren's syndrome can cause complete destruction of any of these areas. Since Sjögren's syndrome can affect the liver and pancreas, there is a greater chance of developing cancer of the lymph tissue. However, this is an unusual and rare result.

Sjögren's syndrome can occur in two ways. It is 'secondary' Sjögren's syndrome when it occurs in people who have a rheumatic (pronounced room-a-tic) condition or connective tissue disease such as lupus (pronounced loo-pus), scleroderma or polymyositis (pronounced pol-ee-my-o-site-iss). It is named 'primary' Sjögren's syndrome when dry eyes and mouth are not associated with a rheumatic condition.

The disease is named after the Swedish eye doctor, Dr. Henrik Sjögren, who first described it in 1933. He had been treating a group of women who had chronic arthritis accompanied by dry eyes and mouth.

Sjögren's syndrome is estimated to affect at least 110,000 Canadians. Some estimates put it as high as 430,000.

Sjögren's syndrome can be found in people of all ages and races. However, 90% of those diagnosed with Sjögren's syndrome are women. Usually it occurs after age 45, which generally coincides with the end of the childbearing years for most women.

About half the people diagnosed with Sjögren's syndrome also have other rheumatic conditions or connective tissue diseases such as rheumatoid arthritis, lupus, scleroderma or polymyositis.

The symptoms of Sjögren's syndrome vary from person to person. The most common early symptoms are dry mouth and eyes. For people who have rheumatoid arthritis or another connective tissue disease, the onset of dry eyes and mouth may develop slowly.

If you have Sjögren's syndrome the reduced amount of saliva in your mouth may make it difficult for you to chew and swallow. Your eyes may feel gritty or sandy. Please note however that having these symptoms does not automatically mean that you have Sjögren's syndrome. Dryness of the eyes and mouth can be a result of other conditions such as hormonal disorders, the use of certain medications or aging.

Other warning signs of Sjögren's syndrome include swollen salivary glands and a dry nose and throat. Sjögren's can cause you to get more cavities in your teeth because your mouth will produce less saliva to wash away germs. In a woman dryness of the vagina can also indicate Sjögren's syndrome.

If you have Sjögren's syndrome you could also have fatigue, joint pain (most often the smaller joints and usually symmetrical - this means affecting the same spot on both sides of the body) and problems in other parts of the body such as the blood vessels, nervous system, muscles, skin and other organs.

There are no universally recognized criteria for the diagnosis of Sjögren's syndrome. Generally a diagnosis is based on the presence of two of three features: dry eyes, dry mouth and a connective tissue disease. Although Sjögren's syndrome is not especially difficult to diagnose, it is not widely known or recognized.

If your doctor thinks you might have Sjögren's syndrome he or she will probably perform a physical examination and order other tests. Your doctor will be looking for symptoms such as a dry cracked tongue, red, itchy eyes, swollen salivary glands, and enlarged lymph glands in your neck. 

A 'Schirmer test' can determine the amount of tears being produced by your tear glands. This test involves putting a small strip of filter paper under the lower eyelid. A test called a slit-lamp examination involves placing a drop of dye into the eye and then examining the eye for areas of dryness or erosion.

A salivary function test may be done to measure the amount of dryness in your mouth. Blood tests that check for specific markers in the blood may also be done, although not everyone with Sjögren's syndrome will have these markers. In rare instances, a certain blood marker often found in women with Sjögren's syndrome can be associated with heart problems in newborn babies. Therefore, if you might have Sjögren's syndrome it is important to discuss the issue of pregnancy with your doctor.

Other tests may include urine tests and chest x-rays. A lip biopsy, where a very small bit of your lip is cut away and examined in a lab, may also be done.

As there is no cure for Sjögren's syndrome, treatment is aimed at preventing damage to the eyes, mouth and other affected areas, reducing the effects of dryness by putting as much fluid back into your system as possible, and reducing any pain you may experience. Since Sjögren's syndrome affects each person differently, your treatment plan will be based on your individual needs. Your active involvement in developing your prescribed treatment plan is essential.

Medicine

NSAIDs reduce pain when taken at a low dose, and relieve inflammation when taken at a higher dose. NSAIDs such as ASA (Aspirin, Anacin, etc.) and ibuprofen (Motrin IB, Advil, etc.) can be purchased without a prescription. Examples of NSAIDs that require a prescription include Naprosyn, Relafen, Indocid, Voltaren, Feldene, and Clinoril. The various NSAIDs and Aspirin®, if taken in full doses, usually have the same levels of anti-inflammatory effect. However, different individuals may experience greater relief from one medication than another. Taking more than one NSAID at a time increases the possibility of side effects, particularly stomach problems such as heartburn, ulcers and bleeding. People taking these medications should consider taking something to protect the stomach, such as misoprostol (Cytotec).

Cortisone is a steroid that reduces inflammation and swelling and that can influence regulation of the immune system. It is a hormone naturally produced by the body. Corticosteroids are man-made drugs that closely resemble cortisone. 

The most common form of corticosteroid is called prednisone, taken in pill form. Prednisone use needs to be carefully monitored because of its many side effects, and the drug must never be stopped abruptly. Some of the side effects from long-term use include cataracts, high blood pressure, sleep problems, muscle loss, bruising, thinning of the bones (osteoporosis), weight gain and increased risk of infections. The goal with this and most drugs is to find the lowest effective dose that will avoid as many of the side effects as possible.

DMARDs target the processes causing the inflammation, but do not reverse permanent damage. The most common of them are gold salts, methotrexate, sulfasalazine, hydroxychloroquinine, chloroquinine and azathioprine. Hydroxychloroquinine is often used to treat Sjögren's syndrome. DMARDs are usually given in addition to other medications such as NSAIDs. They usually take a few months to make a difference in the inflammation. Side effects may include mouth sores, diarrhea and nausea. More serious side effects, monitored through regular blood and urine tests, include liver damage, and excessive lowering of the white blood cell count (increasing susceptibility to certain infections) and platelet count (affecting blood clotting).

Body and skin care

If you have a dry mouth as a result of Sjögren's syndrome, ask your doctor or pharmacist about saliva substitutes or mouth coating products. Also consult your pharmacist about medications that can cause dryness, such as antihistamines and antidepressants.

Be sure to see your dentist regularly as the lack of saliva in your mouth can cause an increase in cavities in your teeth.

If you have dry eyes, talk to your doctor or ophthalmologist about artificial tear preparations or lubricating ointments for overnight or long-lasting relief. If your eyes are severely dry your ophthalmologist can perform a simple operation that blocks tear drainage from the eye. This allows tears that are produced to accumulate and moisten the eyes. Moisture chamber eyeglasses, which preserve existing tears and protect the eyes from wind and draft, can also be useful.

If you have a dry nose, ask your doctor or pharmacist about the different types of nasal preparations available that you can use to keep the area moist.

If you are a woman with Sjögren's syndrome and are experiencing vaginal dryness you should talk to your doctor or pharmacist about specially designed lubricants you can use.

Exercise

Along with the physical symptoms of arthritis, many people experience feelings of helplessness and depression. Learning daily living strategies to manage your arthritis gives you a greater feeling of control and a more positive outlook. To get the best results, people affected by arthritis need to form close ties with their doctors and therapists, and become full partners in their treatment. From our perspective, it's all part of 'living well with arthritis.' There are several resources you can use in finding out how best to manage your own arthritis. Here are a few:

• The Arthritis Self-Management Program (ASMP) is a unique self-help program offered by The Arthritis Society to help you better control and manage your arthritis.
• The Open Forum within this Web site is an opportunity to discuss and share information with other visitors - people who, through their own experiences, may be able to offer some useful insights.

Of course, there are many other valuable resources for people with arthritis. If you're unclear about where to look for help, be sure to call The Arthritis Society at 1-800-321-1433.