Interest in keratoconjunctivitis sicca, which is an inflammation of the cornea and conjunctiva associated with dryness, was limited primarily to ophthalmologists until 1933 when Sjögren1reported finding stomorhinopharyngolaryngitis sicca, enlargement of the salivary glands, and arthritis in patients with keratoconjunctivitis sicca. The association of these conditions with keratoconjunctivitis sicca since then has been designated as Sjögren's syndrome. This study was undertaken to investigate the relationship of other clinical conditions to keratoconjunctivitis sicca and Sjögren's syndrome and to determine the occurrence of various protein and hematologic abnormalities in both conditions. We also wished to determine the incidence of the various manifestations of Sjögren's syndrome among patients with keratoconjunctivitis sicca and to study the course and prognosis of both conditions.
Materials and Methods
The records of 248 patients with keratoconjunctivitis sicca observed at the Mayo Clinic from 1950 through 1956 were reviewed. Each patient was examined by an ophthalmologist, and keratoconjunctivitis
the relationship of other clinical conditions to keratoconjunctivitis sicca and Sj\l=o\gren's syndrome and to determine the occurrence of various protein and hematologic ab- normalities in both conditions. We also wished to determine the incidence of the various manifestations of Sj\l=o\gren'ssyn- drome among patients with keratocon- junctivitis sicca and to study the course and prognosis of both conditions. ) typical symptoms such as stinging, burning, and foreign-body sensation, (2) dryness of the conjunc¬ tiva as indicated by Schirmer's test,2 and (3) corneal and conjunctival staining with Bengal rose or fluorescein typical of keratoconjunctivitis sicca. Sjogren's syndrome was diagnosed when patients had keratoconjunctivitis sicca plus one or more additional features of the syndrome. In considering arthritis we included only joint diseases, such as rheumatoid arthritis, which may be regarded as systemic disorders. The incidence of associated clinical conditions, protein changes, and hematologie abnormalities in cases of Sjogren's syndrome was compared with that in cases of keratoconjunctivitis sicca without other evidence of Sjogren's syn¬ drome. Specimens of bone marrow were examined in 22 cases of Sjogren's syndrome and differential counts of 500 cells were performed. Follow-up letters were sent to the 248 patients in the current study and to 121 additional patients with kerato¬ conjunctivitis sicca who were the subjects of an earlier study.3
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