Related topics: •  Arthritis Center

For years Wendy Stewart, 42, suffered from a variety of symptoms that doctors were unable to diagnose. Her kidneys ached. She had trouble swallowing. Then her eyes, which were usually dry and sensitive to light, began to feel even more tired and gravelly. Her teaching job became trying because it was difficult to talk all day when her mouth always felt parched.

What's more, the symptoms were worsening. Her muscles ached. When she coughed, her chest hurt so much that she was unable to stand. After one bad bout of coughing, she felt so drained she had to call her husband for a ride back to their home in El Sobrante, California.

Stewart's family doctor thought she had bursitis in her left shoulder. She visited another physician who thought the teacher was "just getting old" and prescribed an anti-inflammatory medication. Finally Stewart visited a rheumatologist, a doctor who specializes in arthritis and certain autoimmune diseases. "He took my medical history, and right away he knew [what it was]," she recalls.

The diagnosis was Sjögren's syndrome, a disorder that causes the body's immune system to attack healthy tissue. The condition is named after Swedish doctor Henrik Sjögren, who in 1933 first noticed that many of his patients reported having abnormally dry eyes and mouths in addition to other symptoms commonly associated with arthritis. Like Stewart, many people with the disorder don't know what's wrong with them.

In Stewart's case the condition targeted the glands that produce moisture. If these glands are damaged enough, they will eventually become unable to produce tears and saliva, making it painful to cough or cry, for example. Sjögren's syndrome also affects other glands and organs (especially those that produce sweat and mucus), so your sinuses, lungs, and joints may ache and your skin may feel unusually dry.

Up to 4 million people have Sjögren's syndrome, according to the Sjögren's Syndrome Foundation. However, there may be many more people whose condition is being mistaken for other forms of arthritis, says Stanley R. Pillemer, MD, a rheumatologist and senior staff physician who heads the Sjögren's Syndrome Clinic at the National Institute of Dental and Craniofacial Research in Bethesda, Maryland. About 90 percent of sufferers are women. Doctors aren't sure why women are more susceptible than men, but hormones may have a role, says Pillemer.

People who have Sjögren's syndrome may also have arthritis or other immune diseases, which can make it difficult to get an accurate diagnosis. So if you have one condition that affects your joints, like lupus or rheumatoid arthritis, it's not uncommon to have Sjögren's syndrome as well. In fact, about 10 to 15 percent of people with rheumatoid arthritis, and 20 percent of patients with lupus also suffer from Sjögren's syndrome, according to Pillemer.

"We had a patient who, after 15 years of having Sjögren's syndrome, came down with lupus," says Pillemer. "Some diseases overlap."

Symptoms may also overlap. "Patients with fibromyalgia will also complain of dry eyes and a dry mouth," he says. "First of all they have fatigue and difficulty sleeping." They take antidepressants -- which, he adds, can sometimes cause dry mouth. "The most common cause of dry mouth is medication, and there are more than 400 drugs that will cause dry mouth."

Often Sjögren's syndrome is misdiagnosed as a different type of arthritis-like disorder, and physicians may also mistake it for signs for early menopause. Many people see multiple doctors for up to a decade before receiving a correct diagnosis, Pillemer says.

No one really knows. Some scientists believe that genes play a role. In addition, a serious infection may trigger Sjögren's syndrome in people whose genes may make them vulnerable to the disease. This happens because the cells that are supposed to defend against invading microorganisms don't stop after fighting off the bacteria, and instead attack the body's own tissue.

Your family doctor or specialist can easily diagnose the disease, provided he takes a thorough history of your symptoms. Be sure to mention any relatives with Sjögren's syndrome because there may be a genetic link.

If your physician suspects you have Sjögren's syndrome, you may have to undergo certain tests on your eyes and mouth to determine how dry they are. He or she may also take tiny samples from your salivary glands and tear ducts, and do blood tests to look for the antibodies or lymphocytes that are usually present in people with Sjögren's syndrome. Finally, a doctor may order urinary tests and x-rays to make sure your kidneys and lungs are working properly.

Patients can help take care of themselves by keeping their eyes and mouth moist. You can purchase artificial saliva, saline drops or artificial tears, sugar-free lozenges, and eye ointments over the counter at a pharmacy, but it's important to use eye drops that don't contain preservatives, which can make eyes drier, Pillemer says.

There are several prescription medications that can stimulate your body produce saliva or tears, including pilocarpine, cevimeline, cyclosporine ophthalmic emulsion, and hydroxypropyl cellulose ophthalmic insert. If you have rashes and arthritis, doctors may prescribe an older drug called hydroxychloroquine, which has been used to treat patients with malaria, he says. Because Sjögren's syndrome patients can't produce enough saliva, they may be more likely to develop tooth decay and mouth sores, so people need to be especially careful to watch for dental problems.

People with Sjögren's syndrome are also at higher risk of lymphoma, a type of cancer that affects a person's lymphatic system. People with Sjögren's syndrome are more likely to develop the disease because Sjogren's causes their body to overproduce lymphocytes -- important cells in your immune system, according to Pillemer. With so many of these lymphocytes present, the chance that one of them may transform into a malignant cell increases, he adds.

Stewart's physician monitors her for signs of lymphoma. Meanwhile, she has learned how to curb some of the disease's bothersome symptoms. Among other things, she keeps a bottle of eye drops with her at all times, and drinks plenty of water -- about a cup every hour. "I'm careful about getting dry. I try to drink eight to 10 glasses a day," says Stewart. The only positive thing she can find about having Sjögren's syndrome is that even on the hottest days, she can enjoy herself outside without sweating.

-- Psyche Pascual is the articles editor at Consumer Health Interactive. She is a former staff writer for the Los Angeles Times.

Further Resources

National Institutes of Health, Sjögren's Syndrome http://www.nlm.nih.gov/medlineplus/sjogrenssyndrome.html

Sjögren's Syndrome Foundation http://www.sjogrens.org

Sjögren's Syndrome Clinic http://www.nidcr.nih.gov/Research/NIDCRLaboratories/MolecularPhysiology/SjogrensSyndrome/default.htm

References

Last updated August 11, 2009
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