Sjogren's Syndrome

Description

Sjögren's syndrome is an autoimmune disease, which means that the immune system has mounted an attack against specific tissues of the body. For example, most patients with Sjögren's syndrome carry antibodies to molecules found in the nucleus of cells (antinuclear antibodies). Although Sjögren's syndrome can affect practically any organ in the body, it is characterized by dry mouth (xerostomia) and dry eyes (xerophthalmia). These hallmark symptoms are known as "sicca symptoms." Sjögren's syndrome goes by many names which include Sjögren's disease, dry-mouth and dry-eyes disease, sicca complex, and sicca syndrome.

Symptoms of Sjögren's syndrome include dry mouth, difficulty or inability to swallow (dysphagia), tooth decay (dental caries), impaired taste and smell, dry eyes, eye pain, eye redness, muscle pain (myalgia), and fatigue. Other, less common, symptoms include diarrhea, headaches, joint pain (arthralgia), muscle weakness, and dry cough. Patients with cancer of lymphoid tissue (lymphoma) and Sjögren's syndrome have fever, nerve involvement, low numbers of red blood cells (anemia) and white blood cells (lymphopenia), inflammation of blood vessels of the skin (skin vasculitis), and disease of the lymph nodes (lymphadenopathy) much more frequently than patients with Sjögren's syndrome alone.

The symptoms of Sjögren's syndrome can have a pronounced effect on quality of life. Besides causing discomfort, the symptoms also disrupt sleep, which can have side effects such as fatigue, difficulty concentrating, and depression. Patients with Sjögren's syndrome are at risk for tooth decay and yeast infections in the mouth (erythematous candidiasis). Approximately 5% of the patients with Sjögren's syndrome develop malignant lymphoma.

Causes

The cause of Sjögren's syndrome is unknown, although several viruses are suspected triggers of the autoimmune reaction. The sicca symptoms of Sjögren's syndrome are caused by the invasion and multiplication of white blood cells (lymphocytes) into the salivary glands and tear glands. The lymphocytes destroy the gland tissue and cause the glands to malfunction, reducing the production of tears and saliva. This invasion by lymphocytes, however, does not fully account for the sicca symptoms. Other, as yet unidentified, factors play a role in the development of the sicca symptoms.

Sjögren's syndrome can occur in combination with certain cancers. For more than half of the patients with non-Hodgkin lymphoma, the lymphoma is located in the salivary glands, causing them to malfunction. Graft-vs.-host disease in patients who have undergone bone marrow transplantation can cause eye problems similar to those seen in Sjögren's syndrome. Both chemotherapy and radiation therapy to the head and neck can cause xerostomia.

Treatments

There is no cure for Sjögren's syndrome. Therefore, treatment is aimed at relieving symptoms. Dry eyes may be treated with eye drops and avoidance of drying conditions such as wind, hair dryers, and medications that cause dry eyes (e.g. tricyclic antidepressants). Eyeglasses may protect the eyes from wind. The lower tear ducts may be blocked with silicone plugs (punctal occlusion) to conserve natural tears. Use of humidifiers, both at home and at work, can significantly reduce sicca symptoms. Saliva substitutes and sugar-free hard candies or chewing gum, which stimulate salivation, can reduce sicca symptoms. The drugs pilocarpine and cevimeline can increase salivation. Pain may be relieved by nonsteroidal anti-inflammatory drugs (e.g. Aleve) or other pain medications.

The patient with Sjögren's syndrome should faithfully conduct routine daily oral hygiene consisting of tooth brushing two to three times, flossing once, and utilizing medicated rinses as prescribed by the physician. Fluoride varnishes applied by a dentist and nightly fluo-ride treatments can help to prevent dental caries. Brushing and flossing should be performed carefully to prevent damage to the weakened oral mucosa.

Alternative and complementary therapies

In a controlled clinical study, the herbal vitamin supplement Longo Vital was shown to increase the rate of salivation. Sicca symptoms may be reduced by acupuncture. Papayas contain papain, which is an enzyme that breaks up proteins. Eating papayas, drinking papaya juice, or drinking a solution of crushed papain tablets in water can liquefy thick saliva. Drinking a solution of meat tenderizer (which contains papain) in water is another alternative.

Resources

BOOKS

Cush, John, MD, and Arthur F. Kavanaugh, MD, et al. Rheumatology Diagnosis and Therapeutics. Philadelphia: Lippincott Williams & Wilkins, 1998.

Iwamoto, Ryan R. "Xerostomia." In Cancer Symptom Manage ment, 2nd ed. Yarbro, Connie H., Margaret H. Frogge, and Michelle Goodman, eds. Sudbury, MA: Jones and Bartlett Publishers, 1999.

PERIODICALS

Bell, Mary, et al. "Sjögren's Syndrome: A Critical Review of Clinical Management." The Journal of Rheumatology 26, no. 9 (2001): 2051-9.

Daniels, Troy E. "Evaluation, Differential Diagnosis, and Treatment of Xerostomia." Current Opinion in Rheuma tology 27 (2001): 6-9.

Fox, Robert I., and Paul Michelson. "Approaches to the Treat ment of Sjögren's Syndrome." Current Opinion in Rheumatology 27 (2000): 15-20.

Fox, Robert I. "Update in Sjögren Syndrome." Current Opin ion in Rheumatology 12 (2000): 391-8.

OTHER

MGH Virtual Brain Tumor Center. 22 June 2001 <http://brain.mgh.harvard.edu>.

Belinda Rowland, Ph.D.

Autoimmune disease

—A disease caused by the abnormal presence of antibodies against normal tissues of the body.

Lymphocytes

—White blood cells. Lymphocytes play a vital role in the immune system.

Lymphoma

—Any cancer of the tissues that make up the lymphatic system.

Sicca symptoms

—Dry mouth and eyes.