Sjögren's Syndrome

Sjögren's syndrome is a disease in which the body's natural defense system (immune system) turns against the body's own cells and attacks the body's glands that produce tears and saliva. Sjögren's syndrome is sometimes linked to rheumatic disorders, such as rheumatoid arthritis and lupus. It is 10 times more common in women than in men. Most people with Sjögren's syndrome are from 45 to 50 years of age.

CAUSES

The cause is unknown. It runs in families. It is possible that a trigger, such as a viral infection, can set it off.

SYMPTOMS

The main symptoms are:

  • Dry mouth.

  • Chalky feeling, mouth feels like it is full of cotton.

  • Difficulty swallowing, speaking, or tasting.

  • Prone to cavities and mouth infections.

  • Dry eyes.

  • Burning, itching, feels like sand in the eyes.

  • Blurry vision.

  • Light sensitive.

Other symptoms may include:

  • Skin, nose, and vaginal dryness.

  • Joint pain, stiffness, and muscle pain.

Other organs that may be affected include:

  • Kidneys.

  • Blood vessels.

  • Lungs.

  • Liver.

  • Pancreas.

  • Brain.

DIAGNOSIS

Diagnosis is first based on symptoms, medical history, and physical exam. Tests may also be done, including:

  • Tear production test (Schirmer's test).

  • A thorough eye exam using a magnifying device (slit-lamp exam).

  • Test to see the extent of eye damage using dye staining.

  • Mouth exam to look for signs of salivary gland swelling and mouth dryness.

  • Removal of a minor salivary gland from inside the lower lip to be studied under a microscope (lip biopsy).

  • Blood tests. Routine and special blood studies will be checked. Antibodies that attack normal tissue (autoantibodies) may be present, such as antinuclear antibodies (ANAs), rheumatoid factors, and Sjögren's antibodies (anti-SSA and anti-SSB).

  • Chest X-ray.

  • Urine tests (urinalysis).

TREATMENT

There is no known cure for this syndrome. There is no specific treatment to restore gland secretion, either. Treatment varies and is generally based upon the problems present.

  • Moisture replacement therapies may ease the symptoms of dryness.

  • Nonsteroidal anti-inflammatory drugs (NSAIDs), such as ibuprofen, may be used to treat musculoskeletal symptoms.

  • Corticosteroids, such as prednisone, may be given for people with severe complications.

  • Immunosuppressive drugs may be prescribed to control overactivity of the immune system. In severe cases, this overactivity can lead to organ damage.

  • A surgical procedure called punctal occlusion may be considered. This is done to close the tear ducts, helping to keep more natural tears on the eye's surface.

A small percentage of people with Sjögren's syndrome develop lymphoma. If you are worried that you might develop lymphoma, talk to your caregiver to learn more about the disease and the symptoms to watch.

HOME CARE INSTRUCTIONS

  • Eye care:

  • Use eyedrops as specified by your caregiver.

  • Try to blink 5 to 6 times per minute.

  • Protect your eyes from drafts and breezes.

  • Maintain properly humidified air.

  • Avoid smoke.

  • Mouth care:

  • Sugar-free gum and hard candy can help in some people.

  • Take frequent sips of water or sugar-free drinks.

  • Use lip balm, saliva substitutes, and prescription medicines as directed.

SEEK MEDICAL CARE IF:

  • You have an oral temperature above 102° F (38.9° C).

  • You have night sweats.

  • You develop constant fatigue.

  • You have unexplained weight loss.

  • You develop itchy skin.

  • You have reddened patches on the skin.

FOR MORE INFORMATION

Sjögren's Syndrome Foundation: www.sjogrens.org

National Institute of Arthritis and Musculoskeletal and Skin Diseases: www.niams.nih.gov