Abstract
PURPOSE: To identify the incidence of cataract and the outcomes of cataract surgery in eyes with ocular cicatricial pemphigoid (OCP). METHODS: Phakic eyes were identified from the Systemic Immunosuppressive Therapy for Eye Diseases Cohort Study and followed for the incidence of visually significant cataract defined as: newly reduced visual acuity 20/50 or worse attributed to cataract; and/or incident cataract surgery. Secondarily, all eyes with OCP that underwent cataract surgery and had a year of follow up thereafter, were included in an analysis of visual outcome. RESULTS: Three hundred fifty-five phakic eyes (200 patients) with OCP were at risk. Eighty eyes developed visually significant cataract over 1064 eye years (incidence rate = 7.5%/eye-year, 95% confidence interval [CI] = 5.6 to 10.1). Higher age was associated with increased incidence of cataract (adjusted hazard ratio [aHR] = 4.47; 95% CI, 1.95-10.23 for age 60-75 inclusive and aHR = 8.37; 95% CI, 3.60-19.42 for age > 75, each compared with age <60 years). Seventy-nine eyes of 61 patients were monitored for > = 1 year following cataract surgery. Cataract surgery was associated with an improvement of vision around 4 lines, which was sustained through at least 48 months. Poorer pre-operative visual acuity was associated with poorer long-term visual outcome. CONCLUSIONS: The incidence of cataract was high in this older population. No factors predictive of cataract such as duration of OCP or use of corticosteroids were identified. Visual acuity improved after surgery by a median of 4 lines' gain at one year; poorer long-term outcome among those with initially poorer visual acuity may be secondary to corneal scarring.