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Sjogren's Syndrome

The second most common autoimmune rheumatic disorder after rheumatoid arthritis (RA), Sjogren's syndrome (SS) is characterized by diminished lacrimal and salivary gland secretion (sicca complex). SS occurs mainly in women (90% of patients); its mean age of occurrence is 50.

SS may be a primary disorder or may be associated with a connective tissue disorder, such as RA, scleroderma, systemic lupus erythematosus, or polymyositis. In some patients, the disorder is limited to the exocrine glands (glandular SS); in others, it also involves other organs, such as the lungs and kidneys (extra glandular SS).

Causes

The cause of SS is unknown. Most likely, genetic and environmental factors contribute to its development. Viral or bacterial infection or perhaps exposure to pollen may trigger SS in a genetically susceptible individual.

Tissue damage results from infiltration by lymphocytes or from the deposition of immune complexes. Lymphocytic infiltration may be classified as benign lymphoma, malignant lymphoma, or pseudolymphorna (nonmalignant, but tumor like aggregates of lymphoid cells).

Signs and symptoms

Sjogren's syndrome can be difficult to diagnose because the signs and symptoms are similar to those caused by other diseases and can vary from person to person. In addition, the side effects of a number of medications can mimic some signs and symptoms of Sjogren's syndrome. Still, typical signs and symptoms of Sjogren's include:

• Dry eyes
• Dry mouth
• Swelling
• Difficulty chewing or swallowing
• Dry cough
• Cavities
• Oral yeast infections
• Dry nose, throat and lungs
• Fatigue

Diagnosis

• Medical exam
• Lab tests
• Chest X-rays
• Lip biopsy
• Schirmer test/slit-lamp exam (measures dryness of eyes)
• Urine test (for kidney function)

Treatment

Usually symptomatic, treatment includes conservative measures to relieve ocular or oral dryness.

Symptomatic treatment

Mouth dryness can be relieved by using a methylcellulose swab or spray and by drinking plenty of fluids, especially at meals. New agents for treatment of salivary hypofunction, such as pilocarpine hydrochloride or bromnexine, may be useful. Meticulous oral hygiene is essential, including regular flossing, brushing, and fluoride treatment at home and frequent dental checkups.

Instillation of artificial tears as often as every half hour prevents eye damage (corneal ulcerations, corneal opacifications) from insufficient tear secretion.

CLINICAL TIP Artificial tears, whose drops are thicker and more viscous, require less frequent application but may cause blurring or leave residue on eyelashes.

Some patients may also benefit from instillation of an eye ointment at bed­time, or from twice-a-day sustained­release cellulose capsules.

If an eye infection develops, antibiotics should be given immediately; topical steroids should be avoided.

Other measures

Other treatment measures vary with associated extraglandular findings. Parotid gland enlargement requires local heat and analgesics; arthritis and arthralgias, hydroxychloroquine or nonsteroidal anti-inflammatory drugs; pulmonary and renal interstitial disease, corticosteroids; accompanying lymphoma, a combination of chemotherapy, surgery, and radiation.

Self-care tips

There is no known way to prevent Sjogren's syndrome. However, by following your doctor's recommendations for treatment, you can avoid any serious complications. Keep in mind that drugs that reduce the amount of saliva will make your dryness worse. These include antihistamines and decongestants commonly taken for the common cold.