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The second most common autoimmune rheumatic disorder after rheumatoid arthritis (RA), Sjögren’s syndrome (SS) is characterized by diminished lacrimal and salivary gland secretion (sicca complex). SS occurs mainly in women (90% of patients); its mean age of occurrence is 50.
SS may be a primary disorder or may be associated with a connective tissue disorder, such as RA, scleroderma, systemic lupus erythematosus, or polymyositis. In some patients, the disorder is limited to the exocrine glands (glandular SS); in others, it also involves other organs, such as the lungs and kidneys (extraglandular SS).
The cause of SS is unknown. Most likely, genetic and environmental factors contribute to its development. Viral or bacterial infection or perhaps exposure to pollen may trigger SS in a genetically susceptible individual.
Tissue damage results from infiltration by lymphocytes or from the deposition of immune complexes. Lymphocytic infiltration may be classified as benign lymphoma, malignant lymphoma, or pseudolymphoma (nonmalignant but tumorlike aggregates of lymphoid cells).
Signs and symptoms
About 50% of patients with SS have confirmed RA and a history of slowly developing sicca complex. However, some seek medical help for rapidly progressive and severe oral and ocular dryness, often accompanied by periodic parotid gland enlargement.
Ocular dryness (xerophthalmia) leads to foreign body sensation (gritty, sandy eye), redness, burning, photosensitivity, eye fatigue, itching, and mucoid discharge. The patient may also complain of a film across his field of vision.
Oral dryness (xerostomia) leads to difficulty swallowing and talking; abnormal taste or smell sensation, or both; thirst; ulcers of the tongue, buccal mucosa, and lips (especially at the corners of the mouth); and severe dental caries. Dryness of the respiratory tract leads to epistaxis, hoarseness, chronic nonproductive cough, recurrent otitis media, and increased incidence of respiratory infections.
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