Sjögren's Syndrome

A cell-mediated autoimmune disease that results in inflammation, dysfunction, and destruction of the exocrine glands.

This chronic syndrome is characterized by decreased lacrimal and salivary gland activity (sicca syndrome) and a connective tissue disorder.

Symptoms, Signs, and Diagnosis

The most common signs, xerophthalmia (dry eyes) and xerostomia (dry mouth), are reported in up to 25% of elderly patients. However, most persons with such symptoms have atrophic mucus-producing cells rather than an autoimmune disease. Patients with xerophthalmia report a foreign body sensation or grittiness in the eyes. Patients with xerostomia have a need to drink copious amounts of fluids, a loss of taste, and excessive dental caries. Occasionally, Sjögren's syndrome is accompanied by systemic manifestations, including Raynaud's phenomenon, polyarthritis, interstitial pneumonitis, vasculitis, neurologic and psychiatric manifestations, and loss of other exocrine functions.

The diagnosis is based on the symptoms. The Schirmer test, which uses a small strip of filter paper to measure eye moisture, has been advocated as a test for Sjögren's syndrome; however, this test is not standardized. Sjögren's syndrome may be accompanied by autoantibodies, including anti-Ro and anti-La, in the blood. Although rarely needed, biopsy of the minor salivary gland of the lip can confirm the diagnosis. Patients show evidence of focal or diffuse lymphocytic infiltration of the glands.

Treatment

Treatment is symptomatic. Artificial tears and lemon and glycerin mouth rinse are the standard therapies. For the systemic forms of Sjögren's syndrome, corticosteroids and other immunosuppressant drugs have been tried with varied effectiveness.