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Abstract A 48 year old woman was referred to hospital for buccal discomfort. Physical examination showed a macroglossia and features of xerostomia. She was diagnosed as having primary Sjogren's syndrome [...]

Connective tissue diseases are often associated with impaired secretion from exocrine glands, which secrete through ducts to the outside. Impaired secretion of the lacrimal, or tear, and salivary glands results in dry eyes and mouth. This condition is known as secondary Sjogren's syndrome and occurs with rheumatoid arthritis (RA) or joint inflammation, systemic lupus erythematosus (SLE), a chronic inflammatory disease of connective tissue, progressive systemic sclerosis, a thickening of organs and tissues, and mixed connective tissue diseases. Primary Sjogren's syndrome is not associated with connective tissue disorders. Studies show that the saliva of patients with Sjogren's syndrome has increased levels of anionic (negatively-charged) proteins, which occur more commonly in secondary Sjogren's syndrome and are detected in patients with RA. Saliva was sampled from 32 patients with RA, 10 with SLE, 3 with mixed connective tissue disease, 12 with progressive systemic sclerosis, 2 with primary Sjogren's syndrome, and 4 with Raynaud's syndrome, a disorder characterized by abnormal blood vessel constriction. The findings demonstrate that saliva of patients with connective tissue diseases contained increased amounts of immunoreactive tissue kallikrein, particularly anionic forms due to higher sialic acid content. Tissue kallikreins are enzymes normally present in saliva. Changes were most obvious in patients with SLE. Abnormalities of glycosylation, a chemical reaction involving sugar groups, may be responsible for increased levels of anionic salivary proteins in connective tissue disease. (Consumer Summary produced by Reliance Medical Information, Inc.)