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A. Taich, M. W. Johnson; A Syndrome Resembling Acute Posterior Multifocal Placoid Pigment Epitheliopathy in Older Adults. Invest. Ophthalmol. Vis. Sci. 2007;48(13):4148.
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To describe clinical characteristics and visual and anatomic outcomes of a syndrome clinically similar to acute posterior multifocal placoid pigment epitheliopathy (APMPPE) in older patients.
We retrospectively reviewed medical records, color fundus photographs, and fluorescein angiograms of consecutive patients over the age of 50 who presented with acute-onset scotomata associated with flat, gray-white lesions at the level of the retinal pigment epithelium (RPE) reminiscent of APMPPE.
The cohort included four men and two women with a mean age of 70.6 (range, 58 to 82) years. The acute gray-white lesions were accompanied by vitritis in 3 (50%) patients and by retinal vasculitis in 1 (17%) patient. The disease course was characterized by recurrent episodes in 4 (67%) patients, with initial or eventual bilaterality in all 5 binocular patients (one patient was monocular due to childhood trauma). Acute lesions faded with or without anti-inflammatory therapy, leaving patches of geographic atrophy of the RPE and inner choroid. Laboratory work-ups were unrevealing. Five of six patients were treated with corticosteroids with significant short-term improvement in visual acuity. However, all patients developed progressive geographic atrophy of the RPE and 5 (83%) developed choroidal neovascularization. With a mean (± S.D.) follow-up time of 6.5±5.5 years, the final visual acuity was 20/200 or worse in 9 (82%) of 11 involved eyes.
APMPPE-like syndrome in an older individual carries a potentially poor visual prognosis. In the short-term, aggressive immunosuppression is likely to improve visual functioning, but progression to geographic atrophy and choroidal neovascular membrane formation is the usual outcome.
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