When flu-like symptoms and subretinal inflammation occur concurrently, think APMPPE.
Acute posterior multifocal placoid pigment epitheliopathy (APMPPE) is an immune-mediated chorioretinal disease. Here’s what you need to know about its neurological and other systemic manifestations.
Carlo J. Pelino, OD, FAAO
Joseph J. Pizzimenti, OD, FAAO
Fast Facts and Stats
About 40% of patients with APMPPE report influenza-like symptoms prior to the onset of visual complaints. Like several other inflammatory “White Dot Syndromes” (WDS), APMPPE usually affects young adults. The mean age of onset is 27 years and it affects males and females in equal numbers.
APMPPE usually involves both eyes. Patients typically present with blur, metamorphopsia or scotomas with characteristic fundus findings. While the exact cause is unknown, researchers suspect a viral etiology. Like other conditions with a viral origin, APMPPE can subside without treatment but may recur at any time.
APMPPE may present with anterior segment findings that include episcleritis, non-granulomatous uveitis and perilimbal anterior stromal corneal infiltrates. The vitreous may have a mild cellular reaction that usually accompanies multifocal yellowish-white flat placoid lesions located mainly in the posterior pole (Figure 1). The lesions do not extend beyond the equator and tend to fade over a two-week period, where they are replaced by varying degrees of RPE atrophy and hyperpigmentation.
Other ocular signs may include papillitis, retinal periphlebitis, central retinal vein occlusion, optic nerve neovascularization and subhyaloid hemorrhage. Intravenous fluorescein angiography (IVFA) will show a classic “block early, stain late” pattern. In the early phase the acute lesions will appear hypoflurosecent, suggesting nonperfusion or infarction of the RPE, the choroid or both (Figure 2). The lesions then become hyperfluoresent in the late phase of the angiogram (Figure 3).
OCT findings in APMPPE include subretinal fluid with a hyperreflective line anterior to the RPE.
Several conditions can simulate APMPPE (Table 1). The most similar is serpiginous choroiditis (SC). The chorioretinal lesions in SC are localized to the posterior pole, but produce a more profound choroidal atrophy than in APMPPE. SC is slower to resolve, and patients have a poorer visual prognosis, with more frequent recurrences of inflammation. Similar posterior segment lesions can occur in patients with sarcoidosis, syphilis and tuberculosis; therefore, clinicians should test patients to exclude those conditions.
A review of systems is important in cases of suspected APMPPE. Systemic associations may involve the skin (erythema nodosum), kidneys (nephritis with urine casts), muscles, thyroid gland (thyroiditis) and blood vessels (vasculitis). It has also been associated with multiple complications in the central nervous system (CNS), including cerebral vasculitis, peripheral neuropathy, headaches, aseptic meningitis, meningoencephalitis, sixth cranial nerve palsy, transient hearing loss and cavernous sinus thrombosis. Symptoms of severe headache or meningeal symptoms warrant neuroimaging and further neurologic workup. Research has associated fatalities due to cerebral vasculitis with APMPPE.
Most cases of APMPPE resolve within a few weeks. Because it is generally self-limiting, there is no rationale for treatment if no neurological complication is encountered. The outcome for the visual system without treatment is characteristically good, and a gradual improvement in visual acuity occurs over weeks to months, with most eyes achieving 20/30 or better. However, when central macular involvement is profound, patients may be treated with systemic steroids or biologic agents such as tumor necrosis factor inhibitors (e.g., infliximab) in an effort to preserve visual function to the greatest possible extent. If CNS vasculitis is present, systemic corticosteroid treatment is recommended.
APMPPE is an immune-mediated disease characterized by discrete areas of subretinal inflammation. It may be associated with a number of systemic conditions and thus warrants a detailed systemic workup. Although the disease is self-limiting with a relatively good prognosis, clinicians should consider prescribing systemic steroids or biologics in cases where fixation is threatened, recurrent debilitating cases, or cases involving systemic problems.