Sjogren Syndrome

Sjogren syndrome is a systemic disorder that is characterized by dry eyes (xerophthalmia) and dry mouth (xerostomia).  Athough the exact cause is unknown, many experts believe that it has an autoimmune basis.  The histologic hallmark of Sjogren syndrome is infiltration of lymphocytes of the lacimosalivary glands.  However, nerve connections to these glands are also affected.  Some studies estimate the incidence of Sjogren syndrome as almost 5% of the general population.

Sjogren syndrome mainly affects middle-age women.  Eye symptoms include grittiness, dryness, itching, and a grainy feeling in the eyes.  Oral symptoms include difficulty swallowing, speaking, or eating.

Eye examination may showing redness of the conjunctiva. Diagnosis may be obtained by biopsy of the salivary glands and testing positive for antibodies to anti-SS-a or anti-SS-B antigen.  However, these tests may be positive for many diseases, including Sjogren syndrome.  Patients with Sjogren may also test positive for antinuclear antibody (ANA) and rheumatoid factor (RF).

Sjogren syndrome often has a gradual onset, a variable course, and a broad spectrum of clinical manifestations.  About 50% of patients with Sjogren syndrome also have gastrointestinal symtoms such as reflux, dyspepsia, diarrhea, and constipation.  Anywhere from 30% to 75% of patients with Sjogren syndrome will also have some form of arthralgia such as rheumatoid arthritis.  Some patients with Sjogren’s syndrome with also have autoimmune thyroiditis.  Patients with Sjogren syndrome also have a 40 times increased risk for developing non-Hodgkin lymphoma.

Eye involvement may be evaluated with the Schirmer tear test or the rose bengal test.  The Schirmer tear test involves placing the base of a sterile filter paper strip behind the lower eyelid for five minutes.  If the moistened area measures less than 5 mm, the test is positive and indicative of dry eyes.  The rose bengal test is performed by instilling a dop of 1% rose bengal in the eye.  Excessive eye dryness with cause staining of the devitalized conjunctiva and cornea cells.  A slit lamp microscope is used in conjunction with the rose bengal test.

A salivary gland biopsy taken from the lip may be considered as a diagnostic aid.  A positive biopsy is defined as at least one focus of dense, inflammatory infiltrate containing at least 50 lymphocytes per 4 mm.

There is no known cure at this time for Sjogren syndrome.  Treatment is directed at relieving the symptoms and preventing complications.  Ocular treatment involves the use of lubricants.  Many experts recommend preservative-free eyedrops since they tend to be better tolerated than solutions with preservatives when used on a long-term basis.  Eydrops such as pilocarpine (Salagen) and Restasis have been reported to have some benefit in patients with Sjogren syndrome.

Cases in Point

Dr. Reynard was retained to evaluate the cause and extent of dry eye syndrome in a patient following lasik surgery.

Dr. Reynard was retained to evaluate the cause and extent of dry eye syndrome in a patient exposed to toxic chemicals.

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