Abstract
The purpose of this study was to evaluate potential insights into the pathogenesis of acute posterior multifocal placoid pigment epitheliopathy (APMPPE) using multimodal diagnostic imaging and laboratory evaluation in long-term follow-up. A retrospective, single-center case series was conducted on seven consecutive patients (14 eyes) who were given a diagnosis of APMPPE from March 1, 2011, through June 30, 2019 with at least three months of follow-up. Clinical characteristics (age, symptoms, visual acuity [VA]), laboratory testing including coxsackievirus titers, and multimodal imaging from fundus photography, spectral-domain optical coherence tomography (SD-OCT), fundus autofluorescence (FAF), fluorescein angiography (FA), and indocyanine green angiography (ICG) were analyzed for each patient. The initial median VA was 20/71 and final median VA was 20/22. Coxsackievirus B (CVB) titers were elevated (≥ 1:80) in six of seven patients, with a four-fold increase in convalescent titers seen in two patients suggestive of recent infection. All patients were treated with oral corticosteroids, and five patients underwent corticosteroid-sparing immunomodulatory therapy. Initially, multifocal deep choroidal lesions were observed in the posterior pole corresponding to patches of hypocyanescence on ICG. Overlying retinal pigment epithelium (RPE) disease was observed on FAF, although this finding was not universally observed, suggesting that RPE disease may occur as a sequelae to unchecked choroidal inflammation. SD-OCT architectural changes confirmed outer retina and ellipsoid zone disruption. FA of active lesions showed early hypofluorescence and late hyperfluorescence with surrounding leakage while inactive disease showed areas of staining. Long-term follow-up of multimodal diagnostic imaging in APMPPE revealed that choroidal inflammation likely precedes RPE change and photoreceptor damage. Elevation of coxsackievirus titers with seroconversion may be associated with an infectious trigger in concert with immune-mediated disease in this posterior uveitis syndrome.
Highlights
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare cause of sudden painless vision loss in young healthy adults originally described by Gass in a series of three patients in 1968 [1]
The patient deferred starting immunosuppression and she was continued on a prednisone taper. In this series of patients with APMPPE, multimodal diagnostic imaging was informative in identifying the structural changes that occur during the early phases of the disease and during disease resolution in long-term follow-up
The use of multimodal diagnostic imaging suggest that choroidal inflammation preceded the development of overlying retinal pigment epithelium (RPE) and photoreceptor damage
Summary
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is a rare cause of sudden painless vision loss in young healthy adults originally described by Gass in a series of three patients in 1968 [1]. A viral prodrome has been reported in up to one-third of cases; the precise etiology of this inflammatory condition remains unknown [2]. Viruses such as Coxsackievirus B4 [3] and Adenovirus type 5 [4] have been implicated in association with APMPPE, and vaccinations have been hypothesized as a potential trigger [5, 6]. Corticosteroids or immunosuppressive therapy have recently been advocated to expedite recovery and decrease chorioretinal scarring [12]
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