Sjögren syndrome is a chronic systemic rheumatic disease that is characterized by excessive dryness of the eyes, mouth, and other mucous membranes.
White blood cells damage glands that make moisture, and sometimes other organs can be damaged.
Dry mouth and dry eyes are the hallmark symptoms of this syndrome.
Established criteria may be used to help make the diagnosis, and tests can be done to measure tear and saliva production and assess the presence of abnormal antibodies in the blood.
The cause of Sjögren syndrome is not known, but it is thought to be an autoimmune disorder. In an autoimmune disorder, antibodies or cells produced by the body attack the body’s own tissues.
Sjögren syndrome is most common among middle-aged women. Some people who have it also have other autoimmune disorders, such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, mixed connective tissue disease, Hashimoto thyroiditis, primary biliary cirrhosis, and chronic autoimmune hepatitis.
In Sjögren syndrome, white blood cells attack and injure the glands that make moisture, such as the salivary glands in the mouth and the tear glands in the eyes, resulting in a dry mouth and dry eyes. The skin and other moist surfaces of the body (mucous membranes), such as in the nose, throat, digestive tract, voice box (larynx), windpipe (trachea), airways of the lungs, vulva, and vagina, also can become dry.
Symptoms of Sjögren Syndrome
In some people, only the mouth or eyes are dry (a condition called sicca complex or sicca syndrome). Dryness of the eyes may severely damage the cornea, resulting in a sandy, irritated sensation, and a lack of tears can cause permanent eye damage. Insufficient saliva (xerostomia) in the mouth can dull taste and smell, make eating and swallowing painful, and can cause dental cavities and salivary gland stones. The mouth may also burn, which may sometimes indicate a complicating yeast infection.
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In other people, many organs are affected. Sjögren syndrome can dry out the skin and the mucous membranes lining the nose, throat, digestive tract, voice box, windpipe, airways of the lungs, vulva, and vagina. Dryness of the vulva and vagina can make sexual intercourse painful. Dryness of the trachea can cause cough and problems with how the vocal cords function. Nerve, lung, and other tissues may be affected by the inflammation.
The salivary glands in the cheeks (parotids) become enlarged and slightly tender in about one-third of people. One gland may grow larger than the other, which may indicate a tumor or other disorder.
Joint inflammation (arthritis) occurs in approximately 20% of people, affecting the same joints that rheumatoid arthritis affects, but the joint inflammation of Sjögren syndrome tends to be milder and is usually not destructive.
Lymph nodes may enlarge throughout the body. Lymphomas, which are cancers of the lymphatic system, can develop, and people are at increased risk of developing non-Hodgkin lymphoma compared to people who do not have Sjögren syndrome.
Rashes, kidney damage, lung problems, pancreatitis, and vasculitis are other manifestations of Sjögren syndrome. Raynaud syndrome, in which the fingers suddenly become very pale and tingle or become numb in response to cold or emotional upset, may also occur.
Hair loss (alopecia) may occur.
Fatigue is common in people with Sjögren syndrome.
Diagnosis of Sjögren Syndrome
Established criteria
Tear and saliva tests
Blood tests
Although a sensation of dry mouth or dry eyes is common, a sensation of dry mouth and dry eyes accompanied by joint inflammation, enlarged salivary glands, nerve damage, certain rashes, or kidney problems may indicate that the person has Sjögren syndrome. Established criteria and various tests can help a doctor diagnose Sjögren syndrome and differentiate it from other disorders that can cause similar symptoms.
The diagnosis is based on all of the information doctors gather, including symptoms, physical examination results, and all test results.
Established criteria
Doctors can use an established set of criteria to help them diagnose Sjögren syndrome. Before they can apply the criteria, doctors first ask questions and do a physical examination to determine whether people have eye or mouth symptoms. Doctors can then apply the criteria to people who have at least 1 of these symptoms of dryness in the eyes or mouth:
Eye symptoms: People have persistent, troublesome dry eyes every day for 3 months or more, have a frequent sensation of feeling as though sand or gravel is in the eyes, or use tear substitutes 3 or more times per day.
Mouth symptoms: People have a dry mouth sensation every day for 3 months or use liquids to help them swallow dry food every day.
Once an eye or mouth symptom is diagnosed, doctors use the criteria to determine whether people have other symptoms that support the diagnosis of Sjögren syndrome. Doctors also use the criteria to rule out other disorders a person may have. However, some people who have Sjögren syndrome do not fulfill the strict criteria above for eye or mouth symptoms.
Tear and saliva tests
A doctor can estimate the amount of tears a person produces by placing a filter paper strip under each lower eyelid and observing how much of the strip is moistened (Schirmer test). A person who has Sjögren syndrome may produce less than one-third of the normal amount. An ophthalmologist (a medical doctor who specializes in the evaluation and treatment of eye disorders) can test for damage to the eye’s surface.
More sophisticated tests to evaluate saliva production may be done, and a doctor may order imaging tests or the removal of tissue for examination and testing (biopsy) of the salivary glands.
Blood tests
Blood tests can detect abnormal antibodies, including antinuclear antibodies (ANA), anti-Ro/SSA, anti-La/SS-B, and rheumatoid factor. The erythrocyte sedimentation rate (ESR), a test that measures the rate at which red blood cells settle to the bottom of a test tube containing blood, is elevated in more than 70% of people who have Sjögren syndrome. Approximately 33% of people have a decreased number of red blood cells (anemia), and up to 25% of people have a decreased number of certain types of white blood cells (leukopenia).
Although blood test results can help doctors diagnose Sjögren syndrome, they alone cannot confirm a definite diagnosis because sometimes the abnormalities they detect are present in healthy people or in people who have other disorders.
Treatment of Sjögren Syndrome
Measures to relieve dryness
No cure for Sjögren syndrome is available, but symptoms can be relieved.
Painful, swollen salivary glands can be treated with analgesics and warm compresses. Doctors remove stones in the salivary glands.
Using a vaginal lubricant can very effectively reduce pain caused by sexual intercourse. Moisturizers can be used to relieve dryness of the skin.
Sjögren syndrome that occurs along with other autoimmune diseases, such as lupus, rheumatoid arthritis, and systemic sclerosis, is referred to as secondary Sjögren syndrome. People with secondary Sjögren syndrome receive additional treatment for the other disease.
There is no clearly effective treatment for fatigue in people with Sjögren syndrome.
Prognosis for Sjögren Syndrome
The prognosis is generally good. Overall health and life expectancy are mainly unaffected in people whose only symptoms are dry eyes and mouth.
However, death occassionally occurs if the lungs are very damaged and, rarely, if kidney failure or lymphoma develops.
More Information
The following English-language resource may be useful. Please note that The Manual is not responsible for the content of this resource.
Sjögren's Foundation: Provides information about living with Sjögren syndrome
