Diagnosis

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No single gene, microorganism or hormone is known to cause Sjögren's syndrome. As a result, diagnosis is often difficult. Its symptoms can mimic those of other diseases, and no two people with Sjögren's syndrome have exactly the same set of symptoms or medical history. Moreover, individual symptoms may be reported to different health care professionals—such as dry mouth to the dentist or dry eyes to an optometrist—so the condition often goes undiagnosed. In fact, the average time from the onset of Sjögren's symptoms to diagnosis is over six years.

Once Sjögren's is suspected, a detailed history and physical exam, blood tests for autoantibodies, tests to determine the degree of dry eye and mouth, and sometimes lip biopsies, can point to the presence of Sjögren's.

Symptoms can include:

• Dry mouth. If you have Sjögren's syndrome, you produce far less saliva than normal. This makes chewing, swallowing and speaking difficult, and it may diminish your sense of taste as well as produce halitosis, or bad breath.

• Dry eyes. Your eyes may feel dry and gritty—they may also burn and turn red and be extremely sensitive to sunlight. A thick substance may accumulate in the inner corner of your eyes while you sleep. If not properly treated, Sjögren's syndrome can sometimes lead to ulcers of the cornea. On rare occasions, this can cause blindness.

• Swollen salivary glands. Two sets of major salivary glands produce saliva. They are under your tongue and in the cheeks in front of your ears. They may feel swollen and tender.

• Dental cavities. Saliva fights bacteria and defends against cavities. If you have Sjögren's syndrome, your saliva will be decreased, and your teeth may develop cavities more easily.

• Dry nose, throat and lungs. Your throat feels dry and tickly, and you may have a dry cough, hoarseness, a decreased sense of smell and nosebleeds. Sjögren's can also lead to pneumonia, bronchitis and ear problems.

• Vaginal dryness. Because Sjögren's can affect the mechanisms for secreting vaginal fluids, you may find sexual penetration difficult.

• Fatigue. Sjögren's may cause you to tire more easily or feel an underlying fatigue. Fatigue is also frequently a symptom of other autoimmune disorders, such as rheumatoid arthritis or lupus.

• Joint pain. If the immune system attack spreads to the joints, movement of fingers or other joints can become difficult or painful. Joint involvement may indicate you have another autoimmune disorder, such as rheumatoid arthritis or lupus.

• Other problems. Sjögren's syndrome can affect other parts of the body, such as blood vessels, the nervous system, muscles, skin and other organs. This can lead to muscle weakness, confusion and memory problems, dry skin and feelings of numbness and tingling. Sjögren's syndrome can also affect the liver and spleen. People with Sjögren's also have a 44 percent increased risk of developing lymphoma (cancer of the lymphocytes). Although this is unusual, it is another reason why medical exams and continued follow-up are critical.

With such a broad array of symptoms, in varying severity from patient to patient, diagnosis can be difficult. You and your health care professional may go through several steps before confirming the diagnosis of Sjögren's syndrome.

• Physical examination. Your health care professional will probably ask you to describe your symptoms. He or she will then look for other signs of Sjögren's, such as red, itchy eyes; swollen salivary glands; a dry, cracked tongue; and enlarged salivary glands in your neck. You'll also want to discuss what medications you are taking—both prescription and over-the-counter, because many medications have anticholinergic effects that may cause dryness of the mouth and eyes.

• Blood tests. Antinuclear antibodies (ANA), a group of antibodies that react against normal components of a cell nucleus, may be found in patients with Sjögren's syndrome, as well as the antibodies SSA (or RO) and SSB (or LA). However, not everyone with Sjögren's has these markers, and not everyone with these markers has Sjögren's syndrome.

• Schirmer test. This helps determine how dry your eyes are. It involves placing a small piece of filter paper under your lower eyelid to measure tear production.

• Rose Bengal and Lissamine Green. These dyes are used to detect epithelial defects on the surface of the eye.

• Lip biopsy. In this test, the health care professional removes a few minor salivary glands from inside your lower lip. The tissue is examined under a microscope. The appearance of the tissue helps determine if you have Sjögren's syndrome.

• Salivary function tests. The Parotid Gland Flow measures the amount of saliva you produce over a certain period of time. Salivary Scintigraphy also measures salivary gland function. Sialography is an x-ray of the salivary-duct system.

• Urine tests. These may be done to test your kidney function.

• Chest x-ray. This can help detect inflammation in the lungs.