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 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 Overview of Lymphoma Lymphomas are cancers of lymphocytes, which reside in the lymphatic system and in blood-forming organs. Lymphomas are cancers of a specific type of white blood cells known as lymphocytes. These... read more , 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 Non-Hodgkin Lymphomas Non-Hodgkin lymphomas are a diverse group of cancers of types of white blood cell called lymphocytes. Often, lymph nodes in the neck, under the arms, or in the groin enlarge rapidly and painlessly... read more at 40 times the normal rate.

Rashes, kidney damage, lung problems, pancreatitis Overview of Pancreatitis Pancreatitis is inflammation of the pancreas. The pancreas is a leaf-shaped organ about 5 inches (about 13 centimeters) long. It is surrounded by the lower edge of the stomach and the first... read more , and vasculitis Overview of Vasculitis Vasculitic disorders are caused by inflammation of the blood vessels (vasculitis). Vasculitis can be triggered by certain infections or drugs or can occur for unknown reasons. People may have... read more that causes damage to the peripheral nerves are some of the less common but severe manifestations of Sjögren syndrome. Raynaud syndrome Raynaud Syndrome Raynaud syndrome, a functional peripheral arterial disease, is a condition in which small arteries (arterioles), usually in the fingers or toes, narrow (constrict) more tightly than normal in... read more occurs in about one third of people with Sjögren syndrome.

Hair loss (alopecia) may occur.

Fatigue is common in people with Sjögren syndrome.

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 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. Overall health and life expectancy are mainly unaffected in people whose only symptoms are dry eyes and mouth. However, if the lungs, kidneys, or lymph nodes are damaged by the antibodies, pneumonia Overview of Pneumonia Pneumonia is an infection of the small air sacs of the lungs (alveoli) and the tissues around them. Pneumonia is one of the most common causes of death worldwide. Often, pneumonia is the final... read more , kidney failure Overview of Kidney Failure Kidney failure is the inability of the kidneys to adequately filter metabolic waste products from the blood. Kidney failure has many possible causes. Some lead to a rapid decline in kidney function... read more , or lymphoma Overview of Lymphoma Lymphomas are cancers of lymphocytes, which reside in the lymphatic system and in blood-forming organs. Lymphomas are cancers of a specific type of white blood cells known as lymphocytes. These... read more may result. People who have Sjögren syndrome have a higher risk of ever developing any type of 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 Systemic Lupus Erythematosus (SLE) Systemic lupus erythematosus is a chronic autoimmune inflammatory connective tissue disorder that can involve joints, kidneys, skin, mucous membranes, and blood vessel walls. Problems in the... read more , rheumatoid arthritis Rheumatoid Arthritis (RA) Rheumatoid arthritis is an inflammatory arthritis in which joints, usually including those of the hands and feet, are inflamed, resulting in swelling, pain, and often destruction of joints.... read more , and systemic sclerosis Systemic Sclerosis Systemic sclerosis is a rare, chronic autoimmune connective tissue disorder characterized by degenerative changes and scarring in the skin, joints, and internal organs and by blood vessel abnormalities... read more , 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.

The following English-language resource may be useful. Please note that THE MANUAL is not responsible for the content of this resource.