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JAMA. 1962;182(9):939. doi:10.1001/jama.1962.03050480045012.
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Keratoconjunctivitis sicca is an eye affliction characterized by corneal irritation and a deficiency of lacrimal secretion. Approximately 30 years ago, Henrick Sjörgren, a Swedish ophthalmologist, suggested that the eye symptoms were only part of a specific complex, and thus was born the Sjörgren, or sicca, syndrome. In the 1,000 cases reported since that time, the syndrome has accumulated additional signs, symptoms, and eponyms which relate to almost every organ system and to the work of numerous clinical investigators.

Currently, Sjörgren's syndrome is defined as a systemic malady which is found mostly in postmenopausal females and which is manifested by dryness of the mucous membranes (particularly those of the eye, nose, mouth, pharynx, and vagina), enlargement of the parotid and lacrimal glands, and clinical evidence of rheumatoid arthritis.

However, because of the diversity of symptoms, the etiology of the syndrome, like the sea god Proteus, has remained elusive. An enzyme deficiency


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