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A. D. Birnbaum, F. Oh, H. H. Tessler, D. A. Goldstein; Pediatric Ocular Sarcoidosis. Invest. Ophthalmol. Vis. Sci. 2008;49(13):3898.
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Sarcoidosis is a granulomatous disease that often presents with intraocular inflammation and is rare in children. We will describe pediatric patients diagnosed with sarcoidosis at the Uveitis Service of the University of Illinois.
Medical records of patients seen between 1997-2007 were reviewed to identify those who presented prior to age 17 with ocular inflammation and in whom a diagnosis of sarcoidosis was considered. A point system was used to classify likelihood of sarcoidosis. Both imaging studies and uveitis consistent with sarcoidosis (granulomatous uveitis or chronic iridocyclitis with multifocal choroiditis) received 2 points. 1 point was awarded each for an elevated serum angiotensin converting enzyme (ACE) and lysozyme. Biopsy-proven sarcoid was considered definite. Patients with 4+ points were classified as "presumed" sarcoidosis; those with 3 points were considered "probable" sarcoidosis. Any patient with < 3 points was excluded.
243 children 16 years of age or younger were evaluated between 1997-2007. Sarcoidosis was considered in 20. Two had biopsy proven disease (parotid or vitreous biopsy). Using the point system, 3 had presumed and 6 had probable sarcoidosis. These 11 patients represented just over half of those in whom the disease was initially considered, and 4.5% of the total number of pediatric uveitis patients seen in that time period. Presenting symptoms were redness, photophobia or pain in 7 patients. 1 presented with floaters, and others were identified on screening exams. Mean age was 11.6 years (range 5-16). 7 patients were male. The racial distribution was African American (n=6), Hispanic (n=3), and Caucasian (n=2). Elevated ACE (n=5) and lysozyme (n=6) were seen. Chest imaging was done on 10 patients of the 11 patients; 4 had signs of sarcoidosis. Anterior segment involvement (n=10) was non-granulomatous (n=6), more often than granulomatous (n=4). Multifocal choroiditis was seen in 7 and retinal periphlebitis in 3 patients. Intermediate uveitis was present in 1 patient. 3 patients presented with systemic conditions possibly related to sarcoidosis (pneumonia, asthma, skin rashes, hearing loss).
Ocular sarcoidosis is uncommon in children, even at a tertiary referral center. Patients often present with symptoms of intraocular inflammation but may be asymptomatic. Pulmonary involvement was detected in fewer than half of the patients, despite reports of almost universal lung involvement in this age group. 81% of patients had posterior segment involvement, with choroiditis or granulomatous periphlebitis, which is higher than the approximately quarter to half of adult patients reported in other studies.
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