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salivary Sjögren disease

Monday 5 December 2011

Sjogren’s Syndrome

Digital cases

- HPC:309 : Salivary Sjögren disease with salivary lymphocytic infiltration (Accesory salivary gland biopsy)
- HPC:324 : Salivary Sjögren disease with salivary lymphocytic infiltration (Accesory salivary gland biopsy)

Sjogren’s Syndrome

Sjogren’s syndrome is an autoimmune disorder which results in immunologically-mediated destruction and inflammatory enlargement of the lacrimal and salivary glands. Other exocrine glands throughout the body may be involved as well.

This leads to the development of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) also known as the sicca syndrome or primary Sjogren’s syndrome.

When the keratoconjunctivitis sicca and xerostomia are also associated with another autoimmune disorder, most commonly rheumatoid arthritis, the secondary form of Sjogren’s syndrome exists.

Other possible associated disorders include systemic lupus erythematosus, scleroderma, dermatomyositis, and mixed connective tissue disease.

Sjogren’s syndrome may occur in the presence of BLL. There appears to be no histopathologic difference between BLL occuring alone and that which occurs in association with Sjogren’s syndrome.

Approximately 90% of the time, the disorder occurs in adult females aged 40 to 60 years, and patients typically present with complaints of burning oral discomfort and what is described as a "sandy" sensation in the eyes. Uni- or bilateral salivary gland enlargement, usually the parotid, occurs in approximately 80% of primary and 30 to 40% of secondary cases of Sjogren’s syndrome.

The lymphocytic infiltrate is predominantly of T cell origin, and serologic evidence exists for the presence of B cell dysfunction and possible loss of immunoregulation. Numerous autoantibodies have been indentified, and the two most important serologic markers for Sjogren’s syndrome are anti-ribonucleoprotein antibodies termed anti-SS-A (Ro) and anti-SS-B (La). Of the two, anti-SS-B appears to be more specific for Sjogren’s syndrome. Certain genetic differences have also been noted between primary and secondary Sjogren’s syndrome, such as associations with different HLA types. Various other autoantibodies which have been identified include those to salivary duct cells, gastric parietal cells, smooth muscle mitochondria, and thyroid antigens. These may account for some of the other findings in this disease. Minor salivary gland involvement forms the basis for labial biopsy in the histopathologic diagnosis of Sjogren’s syndrome. The specimens are graded on the basis of lymphocytic infiltrate with focus scores of greater than l focus/4 square mm proposed as diagnostic.

Treatment for Sjogren’s syndrome is primarily symptomatic. In those instances in which persistent complications arise or in which marked glandular enlargement results in cosmetic deformity, excision of the involved gland may be necessary. Of important note is the recognition that Sjogren’s syndrome has been associated with an increased incidence of lymphoid malignancy, particularly of B cell origin. It has been estimated that this risk is increased perhaps 40-fold, and close observation of these patients is warranted.

Etiology

- salivary auto-immune diseases

  • Sjögren disease (Sjogren syndrome)