What is Sjogren's syndrome?

Sjogren's syndrome is an autoimmune disease, characterized by the abnormal production of extra antibodies in the blood that are directed against various tissues of the body. This particular autoimmune illness features inflammation in the glands of the body. Inflammation of the glands that produce tears (lacrimal glands) leads to decreased water production for tears and eye dryness. Inflammation of the glands that produce the saliva in the mouth (salivary glands, including the parotid glands) leads to mouth dryness.

Sjogren's syndrome that involves the gland inflammation (resulting in dryness of the eyes and mouth, etc.), but not associated with a connective tissue disease, is referred to as primary Sjogren's syndrome. Secondary Sjogren's syndrome involves not only gland inflammation, but is associated with a connective tissue disease, such as rheumatoid arthritis, systemic lupus erythematosus, or scleroderma.

What causes Sjogren's syndrome?

While the exact cause of Sjogren's syndrome is not known, there is growing scientific support for genetic (inherited) factors. The illness is sometimes found in other family members. It is also found more commonly in families that have members with other autoimmune illnesses, such as systemic lupus erythematosus, autoimmune thyroid disease, juvenile diabetes, etc. 90% of Sjogren's syndrome patients are female.

What are symptoms of Sjogren's syndrome?

Symptoms of Sjogren's syndrome can involve the glands, as above, but there are also possible affects of the illness involving other organs of the body (extraglandular manifestations).

When the tear gland (lacrimal gland) is inflamed from Sjogren's, the resulting eye dryness can progressively lead to eye irritation, decreased tear production, "gritty" sensation, infection, and serious abrasion of the dome of the eye (cornea).

Inflammation of the salivary glands can lead to mouth dryness, swallowing difficulties, dental decay, gum disease, mouth sores and swelling, stones and/or infection of parotid gland inside of the cheeks.

Other glands that can become inflamed, though less commonly, in Sjogren's syndrome include those of the lining of the breathing passages (leading to lung infections) and vagina (sometimes noted as pain during intercourse).

Extraglandular (outside of the glands) problems in Sjogren's syndrome include joint pain or inflammation (arthritis), Raynaud's phenomenon, lung inflammation, lymph node enlargement, kidney, nerve, and muscle disease. A rare serious complication of Sjogren's syndrome is inflammation of the blood vessels (vasculitis), which can damage the tissues of the body that are supplied by these vessels.

A common disease that is occasionally associated with Sjogren's syndrome is autoimmune thyroiditis (Hashimoto's thyroiditis), which can lead to abnormal hormone levels detected by thyroid blood tests. Heartburn and difficulty swallowing can result from gastroesophageal reflux disease (GERD), another common condition associated with Sjogren's syndrome. A rare disease that is uncommonly associated with Sjogren's syndrome is primary biliary cirrhosis, an immune disease of the liver that leads to scarring of the liver tissue. A small percentage of patients with Sjogren's syndrome develop cancer of the lymph glands (lymphoma). This usually develops after many years with the illness. Unusual gland swelling should be reported to the physician.



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