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Donald W. Park, MD; James C. Folk, MD; Scott M. Whitcup, MD; Timothy D. Polk, MD; Kash Kansupada, MD; Connie Fountain, COT; Jeremiah Brown, MD; Robert B. Nussenblatt, MD

Arch Ophthalmol. 1998;116:1025-1029.

Objective  To characterize a group of phakic patients with idiopathic intermediate uveitis as defined by vitritis, cystoid macular edema, and retinal periphlebitis.

Design  Cross-sectional study.

Participants  Nineteen phakic patients (35 eyes) with vitreous inflammation, cystoid macular edema, and/or retinal periphlebitis of unknown cause.

Intervention  None.

Main Outcome Measures  Best-corrected final visual acuities, standardized clinical examinations, photographic and fluorescein angiographic evaluations, and class I and II HLA analysis on all 19 patients.

Results  Fifteen of the 19 patients were women. The mean age was 38 years, the mean follow-up was 104 months, and the mean duration of symptoms was 154 months. All 35 affected eyes had significant vitritis; 21 eyes (60%) had cystoid macular edema, 21 eyes (60%) had retinal periphlebitis. The median initial visual acuity was 20/30. The median final visual acuity was 20/20 with 32 (91%) of 35 eyes having 20/40 or better visual acuity at the final visit. No patient developed "snowbanks" or evidence of systemic disease, including multiple sclerosis or sarcoidosis, during the follow-up period. There were no statistically significant HLA associations in these patients compared with controls from another study from Iowa, but the Iowa phakic patients with cystoid macular edema did differ from the Iowa patients with pars-planitis at loci HLA-B8, HLA-B51, and HLA-DR2.

Conclusions  We describe a disease entity of idiopathic intermediate uveitis that affects primarily young to middle-aged women and usually causes bilateral vitritis, cystoid macular edema, and retinal periphlebitis. Most patients retained good vision over a prolonged follow-up period. Multiple sequential examinations and HLA associations suggest that these conditions are distinct from other syndromes of intermediate uveitis, particularly pars-planitis.

From the Department of Ophthalmology and Visual Science, University of Iowa Hospitals and Clinics, Iowa City (Drs Park, Folk, Polk, and Brown and Ms Fountain); and the National Eye Institute, Bethesda, Md (Drs Whitcup, Kansupada, and Nussenblatt). Dr Polk is now with The Retina Center, Baltimore, Md.

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