Sjogren’s Syndrome

September 27, 2011

Sjogren’s syndrome (SS) is a chronic autoimmune disorder of unknown cause that mostly involves the tear glands of the eye and salivary glands of the mouth. It can also cause problems in the lining of the genital, urinary, gastrointestinal, and respiratory tracts. Sjogren’s syndrome occurs alone or as part of many other autoimmune disorders. The severity varies considerably. Most Sjogren’s syndrome patients should be treated by specialists in rheumatology, ophthalmology, dentistry, and other necessary specialties. it is estimated that more than 1 million people in the United States suffer from Sjogren’s syndrome, and 90% are women in their 30s and 40s. Connective tissue diseases associated with Sjogren’s syndrome include rheumatoid arthritis, systemic lupus erythematosus, scleroderma, fibromyalgia, and others. Much is still unknown about the cause of Sjogren’s syndrome, but it is clear that infectious, genetic, and hormonal factors are involved.

SYMPTOMS, SIGNS, AND DIAGNOSIS

A health professional examining an individual for symptoms of Sjogren’s syndrome should ask questions about the patient’s eyes and mouth (see Tables 1 and 2).

Table 1. Questions to ask when checking for eye symptoms a
Have you had daily, persistent, troublesome dry eyes for more than 3 months?Do you have a recurrent sensation of gravel or sand in the eyes? Do you use tear substitutes more than three times a day?
a Only one positive response is required to consider the patient symptomatic.

 

Table 2. Questions to ask when checking for oral symptoms a
Have you had a daily feeling of dry mouth for more than 3 months?Have you had recurrently or persistently swollen salivary glands as an adult?

Do you frequently drink liquids to aid in swallowing dry food?

a Only one positive response is required to consider the patient symptomatic.

It also is helpful to note that individuals with Sjogren’s syndrome generally have salivary gland enlargement on both sides although this may not be symmetric. Other diseases that cause salivary gland swelling typically affect only one side.

If eye symptoms are present, certain tests should be performed to evaluate tear production (Schirmer’s test) and quality of tear fluid using microscopic techniques (rose bengal staining). if mouth symptoms are present, objective evidence of salivary gland function is needed. Biopsy examination of the minor salivary glands as well as blood tests must be performed.

To diagnose a patient with Sjogren’s syndrome, it is required that either the biopsy or the blood test results be positive, but even positive results on both tests are not enough to definitely diagnose Sjogren’s syndrome. Mouth and/or eye symptoms must be present as well.

Clinical features of Sjogren’s syndrome are similar to those of several other diseases such as hepatitis C infection, AIDS, preexisting lymphoma, certain autoimmune disorders such as sarcoidosis, and medical complications in transplant patients (graft-versus-host disease). A patient with a past history of head and neck radiation therapy could have symptoms similar to Sjogren’s syndrome as well. Lastly, effects of certain medications (anticholinergic drugs) may mimic Sjogren’s syndrome.

TREATMENT

To treat eye symptoms (keratoconjunctivitis sicca), the patient should be instructed to use artificial tears. Some individuals can benefit from more concentrated viscous solutions or hydroxypropylcellulose pellet insertion under the lower eyelids. Ointments can be helpful as well but should only be used at night because they can interfere with vision. Pilocarpine taken internally has been approved for the treatment of mouth symptoms and can help eye symptoms as well.

Mouth symptoms (xerostomia) require frequent dental care, daily use of antimicrobial mouth rinse, and application of topical fluoride. Artificial saliva and lubricants may relieve oral dryness and help with swallowing difficulty. Some people will find that chewing gum or candies (sugar-free to prevent tooth decay) will stimulate secretion in the mouth. Dietary modifications may aid in swallowing as well. As discussed above, drugs like pilocarpine may increase oral salivary secretion, as long as sufficient gland tissue is present. Oral candidia-sis, a fungal infection, which commonly results from dry mouth, should be treated by having the patient suck oral lozenges or use vaginal suppositories of antifungal agents like nystatin or clotrimazole. Systemic manifestations can be treated by various immune-altering drugs.

PROGNOSIS AND EDUCATION

The prognosis for individuals with Sjogren’s syndrome is usually good. There is, however, an increased risk of progression to non-Hodgkin’s lymphoma. All individuals should be provided with information that will help them cope with the difficulties associated with Sjogren’s syndrome.

See Also: Autoimmune disorders

Suggested Reading

  • Klippel, J. H., et al., (Eds.). (2001). Primer on the rheumatic diseases (12th ed.). Atlanta, GA: Arthritis Foundation.
  • Klippel, J. H., & Dieppe, P. A. (1998). Rheumatology (2nd ed.). St. Louis, MO: C. V. Mosby.
  • Klippel, J. H., Dieppe, P. A., & Ferri, F. F. (2000). Primary care rheumatology. London: Harcourt.

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