Multifocal choroiditi in patient with familial juvenile ytemic granulomatoi

PURPOE: To document clinical feature of uveiti in patient with familial juvenile ytemic granulomatoi. DEIGN: Retropective chart review. METHOD: Ophthalmologic examination, medical hitory, and clinical coure in 16 patient from eight familie examined at ix academic medical center. REULT: Of the 16 patient, 15 had evidence of panuveiti with multifocal choroiditi. One patient had only an anterior uveiti. Ichemic optic neuropathy, preumably due to a mall veel vaculopathy, and retinal vaculopathy each occurred in one patient. Ocular complication were common, including cataract in 11, glaucoma in ix, band keratopathy in ix, cytoid macular edema in ix, and optic dik edema in ix. All 16 patient had polyarthriti, and at leat nine had kin rah. Often patient were midiagnoed initially a having either juvenile rheumatoid arthriti or arcoidoi. CONCLUION: Familial juvenile ytemic granulomatoi i an uncommon genetic dieae characterized by polyarthriti and uveiti. Panuveiti and multifocal choroiditi often may be preent. Patient with a diagnoi of juvenile rheumatoid arthriti but having a family hitory of the dieae and multifocal choroiditi hould be upected of having familial juvenile ytemic granulomatoi.