Sjögren's syndrome is an
autoimmune disorder in which
immune cells attack and destroy the
exocrine glands that produce
tears and
saliva. It is named after Swedish
ophthalmologist Henrik Sjögren (
1899-
1986), who first described it. Sjögren's syndrome is also associated with
rheumatic disorders such as
rheumatoid arthritis, and it is
rheumatoid factor positive in 90 percent of cases. The hallmark symptoms of the disorder are
dry mouth and
dry eyes (part of what are known as sicca symptoms). In addition, Sjögren's syndrome may cause
skin,
nose, and
vaginal dryness, and may affect other
organs of the body, including the
kidneys,
blood vessels,
lungs,
liver,
pancreas, and
brain. Nine out of ten Sjögren's patients are women and the average age of onset is late 40s, although Sjögren's occurs in all age groups in both women and men. It is estimated to strike as many as 4 million people in the United States alone making it the second most common autoimmune rheumatic disease.
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The syndrome of dry eyes (keratoconjuntivitis sicca) in the absence of rheumatoid arthritis or any of the autoimmune diseases is known as 'primary sjogren's syndrome'. There is an association with HLA B2 DR3. Dryness of mouth, skin or vagina may also be a problem. Salivary and paroyid gland enlargement is seen. Associated systemic features include:
*arthralgia and occasional non-progressive polyarthritis, like that seen in SLE (but much less common)
*Raynaud's phenomenon
*dysphagia and abnormal oesophageal motality as seen in systemic sclerosis (but less common)
*other organ-specific autoimmune diseases, including thyroid disease, myasthenia gravis, primary biliary cirrhosis and autoimmune hepatitis
*renal tubular defects (uncommon) causing nephrogenic diabetes insipidus and rena tubular acidosis
*pumonary diffusion defects and fibrosis
*polyneuropathy, fits and depression
*vasculitis
*increase incidence of non-Hodgkins B cell lymphoma.