Faculty Bibliography

PURPOSE: To evaluate the rate of Sjogren syndrome (SS) in a cohort of patients with dry eye syndrome. METHODS: Medical records of patients with a primary diagnosis of dry eye syndrome (International Classification of Diseases [ICD] code 375.15 or 370.33) were reviewed retrospectively. Patients who had 2 or more visits to a single dry eye center during a 2-year period (January 2004 to January 2006) were considered. RESULTS: Two hundred twenty patients with dry eye syndrome were identified. A total of 57 patients (25.9%) had an underlying rheumatic condition: 25 patients (11.4%) had rheumatoid arthritis and 24 (10.9%) had primary Sjogren syndrome (PSS). Majority of the patients with rheumatoid arthritis (96%) carried the diagnosis at the time of presentation. Of all patients with PSS, only 33.3% (8/24) carried the diagnosis at the time of presentation. Fifty percent (12/24) were diagnosed as a result of the initial evaluation. Among those, only 66.6% (8/12) tested SSA (anti-Ro antibodies) or SSB (anti-La antibodies) positive. One third of patients (4/12) tested only antinuclear antibody positive at a titer of <1/320 and required minor salivary gland biopsy for definitive diagnosis. Additional 16.7% (4/24), who were initially serologically negative, eventually underwent minor salivary gland biopsy and became diagnosed with SS. CONCLUSIONS: PSS seems to be underdiagnosed in patients with dry eye syndrome and should be the focus of diagnostic evaluations. A minor salivary gland biopsy might be required for a definitive diagnosis in a significant proportion of the patients with SS.

An 80-year-old woman presented with signs and symptoms consistent with bilateral nasolacrimal duct obstruction. She also was noted to have multiple eyelid margin lesions. She underwent bilateral dacryocystorhinostomy with biopsy of the nasolacrimal ducts and eyelid lesions. Her tearing resolved. Histopathology of the eyelid lesions was consistent with lipoid proteinosis. Nasolacrimal duct obstruction may occur in patients with lipoid proteinosis (Urbach-Wiethe syndrome).