White blood cells can infiltrate and damage glands that secrete fluids, and sometimes other organs can be damaged.
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.
Usually, measures to keep surfaces such as the eyes and mouth moist are sufficient, but sometimes hydroxychloroquine or methotrexate are helpful for problems with the skin or joints.
Sometimes, when internal organ damage is severe, corticosteroids or, for certain symptoms, rituximab can be given.
Sjögren syndrome is thought to be an autoimmune disorder, but its cause is not known. It is most common among middle-aged women. Some people with Sjögren syndrome also have other autoimmune disorders, such as rheumatoid arthritis, systemic lupus erythematosus, systemic sclerosis, vasculitis, mixed connective tissue disease, Hashimoto thyroiditis, primary biliary cirrhosis, and chronic autoimmune hepatitis.
White blood cells infiltrate the glands that secrete fluids, such as the salivary glands in the mouth and the tear glands in the eyes. The white blood cells injure the glands, resulting in a dry mouth and dry eyes—the hallmark symptoms of this 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 scratchy or irritated sensation, and a lack of tears can cause permanent eye damage. Insufficient saliva in the mouth can dull taste and smell, make eating and swallowing painful, and can cause dental cavities and salivary gland stones. The salivary glands in the cheeks (parotids) become enlarged and slightly tender in about one third of people. The mouth may also burn, which may sometimes indicate a complicating yeast infection.
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 (larynx), windpipe (trachea), 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. Nerve, lung, and other tissues may be affected by the inflammation.
Joint inflammation (arthritis) occurs in about one third 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. Lymphoma, a cancer of the lymphatic system, is more common among people who have Sjögren syndrome than the general population, and people develop non-Hodgkin lymphoma at 40 times the normal rate. Rashes, kidney damage, lung problems, pancreatitis, and vasculitis that causes damage to the peripheral nerves are some of the less common but severe manifestations of Sjögren syndrome. Raynaud syndrome occurs in about one third of people with Sjögren syndrome.
Hair loss (alopecia) may occur.
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.
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.
The amount of tears produced can be estimated 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 can test for damage to the eye’s surface.
More sophisticated tests to evaluate saliva production may be done, and a doctor may order scans or the removal of tissue for examination and testing (biopsy) of the salivary glands.
Blood tests can detect abnormal antibodies, including SS-A, an antibody that is present in people who have Sjögren syndrome. Antinuclear antibodies (ANA, which are found in people with lupus) and rheumatoid factor (which is found in people with rheumatoid arthritis) can also be found in people with Sjögren syndrome. 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 about 70% of people. About 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 of Sjögren syndrome because sometimes the abnormalities they detect are present in healthy people or in people who have other disorders. The diagnosis of Sjögren syndrome is based on all of the information doctors gather, including symptoms, physical examination results, and all test results.
The prognosis is generally good. However, if the lungs, kidneys, or lymph nodes are damaged by the antibodies, pneumonia, kidney failure, or lymphoma may result. People who have Sjögren syndrome have a slightly higher risk (about 1 in 10 to 1 in 20 people) of ever developing lymphoma than people who do not have Sjögren syndrome.
No cure for Sjögren syndrome is available, but symptoms can be relieved.
Dry eyes can be treated with artificial tear drops during the day and a lubricating ointment at night. A prescription eye drop containing cyclosporine can also be used. Shields can be fitted on the sides of glasses, helping to protect the eyes from air and wind, reducing evaporation of tears. A simple surgical procedure called punctal occlusion can be performed. In this procedure, an ophthalmologist inserts small plugs into the tear ducts in the corner of the lower eyelid, so the person’s tears stay on the eye longer.
A dry mouth can be moistened by continuously sipping liquids, chewing sugarless gum, or using a saliva substitute mouth rinse. Drugs that reduce the amount of saliva, such as decongestants, antidepressants, and antihistamines, should be avoided because they can worsen the dryness. The drugs pilocarpine or cevimeline may help stimulate the production of saliva if the salivary glands are not too severely damaged. Very careful dental hygiene and frequent dental visits can minimize tooth decay and loss.
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.
Antimalarial drugs (such as hydroxychloroquine) may be given to treat joint symptoms. The drug methotrexate may also be given (alone or in combination with antimalarial drugs) when antimalarial drugs do not successfully relieve joint symptoms.
When symptoms resulting from damage to internal organs are severe, corticosteroids (such as prednisone) or rituximab may be given.
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.