A mouth that feels like it's full of cotton or eyes that feel gritty and burn. For many people with Sjögren's syndrome, these are the extent of their symptoms. But for others, dry mouth and eyes are just the beginning.

A disease in which the immune system attacks and damages the body's moisture-producing glands, Sjögren’s can cause dryness of skin and mucous membranes. Dry skin may itch and crack. Dry vaginal tissues may make intercourse painful. A dry throat may make it hard to swallow.

Sjögren’s can also affect other parts of the body. Patients may experience pain, stiffness and swelling in the joints, rashes on the arms and legs related to vasculitis – an inflammation of tiny blood vessels. The lungs, liver and kidneys may become inflamed; some people develop tingling and numbness in the limbs because of neurological involvement.

The syndrome was identified in 1933 by Swedish ophthalmologist Henrik Sjögren, who observed that a large number of his female patients were experiencing dry eyes and mouths, along with their arthritis symptoms. That Dr. Sjögren noticed more women with the condition is not surprising – of the 1 to 4 million people in the U.S. who have the disease, 90 percent are women. Most develop it after age 40.

The disease is classified either as primary Sjögren’s, meaning it exists entirely by itself, or secondary Sjögren’s, meaning it can develop in conjunction with another connective tissue disorder such as rheumatoid arthritis (RA) or lupus.

Sjögren’s Symptoms and Signs

In addition to dryness of the eyes and mouth, which are known as sicca syndrome, and are the most common indicators of Sjögren’s, symptoms can occur in any part of the body. Here are a few more to look for:

Eyes

• Itching eyes
• Feeling something is in the eye

Mouth and throat

• Difficulty swallowing or eating
• Loss of sense of taste
• Difficulty speaking
• Thick or stringy saliva
• Mouth sores or pain
• Hoarseness

Systemic

• Fatigue
• Fever
• Change in color of hands or feet
• Joint pain or swelling
• Swollen glands

How is Sjögren’s Diagnosed?

Diagnosing Sjögren’s syndrome is often no easy matter. The average time between onset of symptoms and diagnosis is six years. Symptoms rarely develop rapidly, but tend to evolve over a period of months or years, and are not always present when the patient first visits a rheumatologist or other healthcare provider.

In a person who already has a form of arthritis, the sensation of dry eyes and mouth are usually enough to prompt testing for Sjögren’s. However, other diseases and conditions can cause these feelings, particularly in the mouth, and must be ruled out.

To begin the diagnostic process, your doctor will ask questions about the length of symptom duration – dry, gritty eyes or dry mouth and swollen salivary glands lasting more than three months are a sign of Sjögren’s.

Next come physical tests. The Schirmer test measures tear production to determine if your eye creates enough tears to keep it moist. The doctor places a paper strip inside each lower eyelid for five minutes (both eyes are tested at the same time) and the eyes are closed. When time is up, the amount of moisture is measured – 10 mm is considered normal

The slit-lamp exam allows your doctor to check the surface of the eye (cornea) for dryness-related damage. He’ll administer a dye to stain the tear film, most likely via eye drops, and he may dilate your eyes as well. Then he’ll view it through the slit lamp – a microscope with a high-intensity light source.

Oral tests include measuring saliva output, which is accomplished by weighing a dry sponge, inserting it under the tongue and weighing it again to determine how much saliva it absorbed. Salivary scintigraphy, a nuclear imaging test, can assess function of the salivary glands, as can sialography, an X-ray of the salivary glands (because Sjögren’s can affect the lungs, your doctor may order a chest X-ray to check for inflammation). A salivary gland biopsy can be used to collect tissue samples to check for inflammation.

Your doctor may also order bloodwork to check for autoantibodies – certain immune proteins that mistakenly target and damage organs or tissues. Among these are anti-nuclear antibodies (ANAs), Rheumatoid Factor, anti-Ro (SS-A) and anti-La (SS-B).

Your doctor may also test for immunoglobulins, other proteins that participate in ummune reactions and are usually elevated in Sjögren’s patients. And he may measure your Erythrocyte Sedimentation Rate (ESR), the rate at which red blood cells fall to the bottom of a test tube – also an indication of inflammation.

Once all the information is collected, your doctor will determine how many of each criteria you have, and decide if it adds up to a Sjögren’s diagnoses.

Sjögren’s Outlook and Treatment

Although it's frightening to think Sjögren’s can extend beyond the eyes and mouth to many parts of the body, it's important to realize that Sjögren's-related problems can be controlled, if detected early and treated properly, says Robert I. Fox, MD, a leading Sjögren's expert and practicing rheumatologist at Scripps Memorial Hospital in La Jolla, Calif.

While a diagnosis of Sjögren’s syndrome is not life-threatening, like most autoimmune conditions, there is no cure. Current treatments for the condition focus on managing the symptoms: moisture replacement therapies relieve dryness and nonsteroidal anti-inflammatory drugs (NSAIDs) control the inflammation. Those with severe cases may receive corticosteroids, which mimic hormones that fight inflammation in the body, or disease-modifying antirheumatic drugs (DMARDs), which suppress the body’s immune response.

As with other autoimmune diseases like rheumatoid arthritis, the severity of Sjögren’s varies from person to person. Many patients have a mild disease that only affects the eyes and mouth. Others have severe symptoms and still others have symptoms that wax and wane in severity, or may even go into remission.

If you've been diagnosed with Sjögren's syndrome, it's critical that you pay attention to your body and speak to your doctor about the need for regular exams. In addition to your regular primary care physician, ophthalmologist and dentist, you should add a rheumatologist a doctor who specializes in treating –arthritis and related diseases – to your healthcare team.