PHAKIC PATIENTS WITH CYSTOID MACULAR EDEMA, RETINAL PERIPHLEBITIS, AND VITREOUS INFLAMMATION

Objective: To characterize a group of phakic patients with idiopathic intermediate uveitis as defined by vitritis, cystoid macular edema, an d 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. Interve ntion: None. Main Outcome Measures: Best-corrected final visual acuiti es, standardized clinical examinations, photographic and fluorescein a ngiographic evaluations, and class I and II HLA analysis on all 19 pat ients. Results: Fifteen of the 19 patients were women. The mean age wa s 38 years, the mean follow-up was 104 months, and the mean duration o f symptoms was 154 months. All 35 affected eyes had significant vitrit is; 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 o r better visual acuity at the final visit. No patient developed ''snow -banks'' or evidence of systemic disease, including multiple sclerosis or sarcoidosis, during the follow-up period. There were no statistica lly significant HLA associations in these patients compared with contr ols from another study from Iowa, but the Iowa phakic patients with cy stoid macular edema did differ from the Iowa patients with pars-planit is at loci HLA-B8, HLA-B51, and HLA-DR2. Conclusions: We describe a di sease entity of idiopathic intermediate uveitis that affects primarily young to middle-aged women and usually causes bilateral vitritis, cys toid macular edema, and retinal periphlebitis, Most patients retained good vision over a prolonged follow-up period. Multiple sequential exa minations and HLA associations suggest that these conditions are disti nct from other syndromes of intermediate uveitis, particularly parspla nitis.