Acute posterior multifocal placoid pigment epitheliopathy (APMPPE): final outcome and visual prognosis

Abstract

Abstract: : Purpose: To describe thirteen new cases of acute posterior multifocal placoid pigment epitheliopathy (APMPPE). The study includes a review of the literature with the proposal of different treatment options. Methods: We reviewed the clinical records of a consecutive series of patients with APMPPE. Charts were reviewed for age, gender, preceding viral prodromes, visual acuity, ophthalmologic examination findings, CNS findings, and treatment. We used a computerized MEDLINE literature search to identify all references examining prognosis and treatment of APMPPE. Results: Twenty–four eyes of 13 patients were diagnosed with APMPPE. Mean age was 28.1 years (range: 21 to 40 years). Seven patients were males and six females. Final visual acuity was below 20/200 in one eye. Although the final visual acuity was overall good, 70% of eyes were symptomatic with various degree of blurred vision, methamorphopsia, and scotoma. One patient presented CNS involvement. Oral corticosteroid administration did not seem to affect positively the visual prognosis. Conclusions: The analysis of our series of patients and of the literature suggests that the long–term visual outcome following an acute episode of APMPPE may not be as favorable as initially reported. In fact APMPPE can be associated with permanent visual deficits and CNS involvement.. As already proposed for patients with CNS involvement, an aggressive treatment with intravenous corticosteroids should be considered for all patients with APMPPE. The evaluation of different ways of administration of corticosteroid at least for eyes with initial foveal involvement remains necessary. A multicenter study is advised to refine our knowledge on the therapy.

Keywords: choroid • autoimmune disease • uveitis–clinical/animal model