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Sjögren's Syndrome

Sjögren's syndrome is characterized by lymphocytic infiltration of the exocrine glands resulting in xerostomia and dry eyes. It is a chronic, gradually progressive autoimmune disease. Approximately one-third of patients present with systemic manifestations. A small but significant number of the patients may develop malignant lymphoma. The disease can be seen alone (primary Sjögren's syndrome) or in association with silicone.

Incidence and Prevalence

The disease affects predominantly middle-aged women (female-to-male ratio 9:1), although it can be seen in all ages, including childhood. The prevalence of primary Sjögren's syndrome is approximately

Pathogenesis

Sjögren's syndrome is characterized by lymphocytic infiltration of the exocrine glands and B lymphocyte hyperreactivity, as illustrated by circulating autoantibodies.

Sera of patients with Sjögren's syndrome often contain a number of autoantibodies directed against non-organ-specific antigens such as immunoglobulins (rheumatoid factors) and extractable nuclear and cytoplasmic

Phenotypic and functional studies have shown that the predominant cell infiltrating the affected exocrine glands is the helper/inducer T cell with characteristics of memory cells. Both B and T infiltrating lymphocytes.

Immunogenetic studies have demonstrated that HLA-B8, -DR3, and -DRw52 are prevalent in primary Sjögren's syndrome patients as compared with the normal control population. Molecular analysis of HLA class II genes has revealed.

Clinical Manifestations

The majority of the patients with Sjögren's syndrome have symptoms related to diminished lacrimal and salivary gland function. In most patients, the primary syndrome runs a slow and benign course. The initial manifestations can be

The differential diagnosis of Sjögren's syndrome includes other conditions that may cause dry mouth or eyes or parotid salivary gland enlargement. Human immunodeficiency virus (HIV) infection and sarcoidosis appear to produce a clinical picture indistinguishable from Sjögren's syndrome.

Treatment

Sjögren's syndrome remains fundamentally an incurable disease. Treatment of Sjögren's syndrome consists of symptomatic relief and limitation of the damaging

The sicca complex is treated with fluid replacement supplied as often as necessary. To replace deficient tears, there are several readily a

Bromhexine (48 mg/day) and pilocarpine (5 mg thrice daily) given orally appear

Hydroxychloroquine (200 mg/day) is helpful for arthralgias and partially corrects

Glucocorticoids (1 mg/kg per day) or other immunosuppressive agents (i.e.,