What is Sjögren's syndrome? |

Causes and Symptoms

Sjögren’s (pronounced SHOW-grins) syndrome is a chronic autoimmune
disease in which the body’s own immune cells attack and eliminate the glands that produce tears and saliva. This results in dryness of the eyes and mouth and is referred to as sicca syndrome. The causes of Sjögren’s syndrome are not known, although evidence suggests that viral infection, heredity, and hormones may be involved. Sjögren’s syndrome is one of the more prevalent autoimmune disorders. Nine of ten patients with Sjögren’s syndrome are female.

Sjögren’s syndrome can be difficult to diagnose because the symptoms are similar to those caused by other diseases. The symptoms can also mimic the side effects associated with a number of medications and may vary from individual to individual. Even when the symptoms are reported to a physician, dentist, or eye specialist, the proper diagnosis can be overlooked.

The classic symptoms are dry eyes
(xerophthalmia) and dry mouth
(xerostomia). Individuals with Sjögren’s syndrome often have blurred vision, constant eye discomfort, recurrent mouth infections, swollen parotid (salivary) glands, hoarseness, and difficulty in swallowing and eating. Dryness of other mucous membranes of the body, such as the intestines, lungs, and reproductive system, may also occur. Extreme fatigue can also seriously alter the quality of life.

Sjögren’s syndrome is most commonly diagnosed in people in their mid-forties. In some individuals, primary Sjögren’s syndrome affects only the tear ducts and salivary glands. In other patients, it is present in conjunction with other diseases such as rheumatoid arthritis, systemic
lupus erythematosus, systemic sclerosis (scleroderma), or polymyositis/dermatomyositis (secondary Sjögren’s syndrome).

Treatment and Therapy

Once Sjögren’s syndrome is suspected, blood tests for autoantibodies against nuclear or cytoplasmic proteins may be performed. Schirmer’s test, which measures tear production, and salivary scintigraphy, which determines salivary gland function, may also be performed. A lower lip biopsy, to determine the extent of inflammation, may also be needed.

Moisture replacement therapies are designed to ease the symptoms of dryness. The routine use of eyedrops aids in controlling dryness of the eyes, and saliva-stimulating drugs and salivary packets help with difficulties in chewing and swallowing food. For individuals with more severe complications, immunosuppressive or nonsteroidal anti-inflammatory drugs (NSAIDs) may be prescribed.

Perspective and Prospects

Sjögren’s syndrome is named after the Swedish eye doctor Henrik Sjögren, who first identified the syndrome in 1933. There is no known cure for Sjögren’s syndrome, nor is there a current treatment to restore gland secretion. The outlook for individuals with this condition is usually good because Sjögren’s syndrome is generally not life-threatening.

Bibliography

Carson-DeWitt, Rosalyn. "Sjogren's Syndrome." Health Library, Dec. 30, 2011.

Parker, James N., and Philip M. Parker, eds. The Official Patient’s Sourcebook on Sjögren’s Syndrome. San Diego, Calif.: Icon Health, 2002.

Rose, Noel R., and Ian R. Mackay, eds. The Autoimmune Diseases. 4th ed. St. Louis, Mo.: Academic Press/Elsevier, 2006.

"Sjogren's Syndrome." MedlinePlus, May 22, 2013.

Teitel, Ariel D., and David Zieve. "Sjogren Syndrome." MedlinePlus, June 28, 2011.

Wallace, Daniel J., et al., eds. The New Sjogren’s Syndrome Handbook. 3d rev. ed. New York: Oxford University Press, 2005.

"What Is Sjögren's Syndrome?" National Institute of Arthritis and Musculoskeletal and Skin Diseases, July 2010.