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F. S. Oh, A. D. Birnbaum, J. Kaufman, D. E. Choi, H. H. Tessler, D. A. Goldstein; Clincal Features and Diagnostic Evaluation of Biopsy-proven Ocular Sarcoidosis. Invest. Ophthalmol. Vis. Sci. 2009;50(13):6190.
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Sarcoidosis is an idiopathic inflammatory condition with ocular involvement in 25-50% of patients. Definitive diagnosis requires histological evidence of noncaseating granulomas. In ocular sarcoid, biopsy is not always feasible and a clinical diagnosis may be suspected in patients with granulomatous inflammation, elevated angiotensin converting enzyme (ACE) or lysozyme, or characteristic chest imaging. We present the clinical features and diagnostic evaluation of patients with biopsy-proven ocular sarcoidosis.
Patients with biopsy-proven sarcoidosis with intraocular involvement evaluated by the uveitis service from 1997-2007 were identified. Information was collected on patient demographics, clinical findings and diagnostic testing; data was analyzed by race and age.
Thirty-four patients fulfilled the criteria. The most common site for biopsy was lung tissue (41%). The average age of patients was 45.9 years (range 20-84). 70.6% of patients were female; racial distribution included African American (67.7%), Caucasian (29.4%) and other (2.9%). Over 95% of the African Americans presented prior to age 50 years versus 30% of Caucasians. Laboratory results were available in 12 patients. ACE was elevated in 45.5% (5/11) and lysozyme in 57.1% (4/7) with 8/12 patients having at least one elevated serum marker. Non-granulomatous anterior uveitis was identified in one-third of patients regardless of race. Granulomatous iridocyclitis was seen in 47.1% of patients. Over 50% of African Americans presented with granulomatous anterior uveitis versus 30% of Caucasians. Choroiditis was seen in 29.4% of all patients, but was more common in Caucasians (50%) than African Americans (21.7%). Intermediate uveitis was seen in 11.8% of patients and vasculitis in 5.9% of patients. Chest x-ray results were available for 16 patients; 11 (68.8%) had findings consistent with sarcoid. Chest CT results were available for 7 patients; 6 (85.7%) were positive for sarcoid.
Ocular sarcoid is typically associated with granulomatous inflammation, but almost 1/3 of our biopsy-proven patients had non-granulomatous disease. Interestingly, granulomatous inflammation was more typical of African Americans, and choroiditis was more common in Caucasians. African Americans also presented at a younger age than Caucasians. More than 30% of patients with biopsy-proven sarcoidosis did not have elevated serum ACE or lysozyme, thus normal levels should not be interpreted as evidence against the diagnosis of sarcoidosis.
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