The disease is more frequent in young healthy adults between the 2nd to 4th decades and can be preceded by a flu-like illness.
APMPPE affects both the retina and the choroid, classically manifesting with multiple, placoid, yellow-white lesions located deep in the retina in the central part of the eye, the posterior pole.
The anterior part of the eye is usually not involved.
Mild inflammation of the vitreous (the jelly-like structure which fills the posterior cavity of the eye) which is known as vitiritis, and inflammatory involvement of the retinal vessels and optic nerve can be detected.
The lesions gradually fade over 1-2 weeks, but new lesions can appear up to few weeks after the onset.
Older lesions result in loss of tissue (chorioretinal atrophy) and hyperpigmentation.
Extra ocular involvement can be present in form of neurological symptoms due to inflammatory involvement of cerebral vessels (cerebral vasculitis).
The exact pathogenesis of APMPPE is unknown.
The inflammatory damage occurs primarily at the level of the vessels of the inner choroid, with secondary retina involvement.
An infectious trigger is thought to be involved in the pathogenesis of the disease, since patients frequently report viral symptoms before the ocular involvement.
Symptoms include a sudden, painless, decrease in vision, manifesting as photopsias (flashing lights) and scotoma (area of alteration in the field of vision consisting of partially diminished or totally degenerated visual acuity).
Symptoms are usually bilateral but can occur several days apart.
There are no diagnostic laboratory tests and the clinical presentation of APMPPE is usually sufficient to confirm the diagnosis.
Dye tests, including fundus fluorescein angiography and indocyanine green angiography, are sometimes carried out to confirm the clinical suspicion.
The retinal function can be measured by special tests, including visual fields and electrodiagnostic tests.
Central nervous system imaging, such as brain magnetic resonance, can be performed to rule out cerebral vasculitis.
The disease is usually self-limited and resolves spontaneously over a period of weeks. However, steroids are usually administered to hasten visual recovery, especially in cases with macular involvement.
Recurrent or chronic APMPPE with high recurrence rate and long period of disease activity may require the addition of immunosuppressive drugs.
Cerebral vasculitis will be treated by a neurologist and involves the use of systemic steroids and in many cases, immunosuppressive drugs.
APMPEE has a generally good prognosis, with visual recovery typically taking a few weeks up to months. However, in case of foveal involvement visual outcome is significantly compromised.
Abnormal vessels growing under the retina (choroidal neovascularization) is a common complication that can affect the quality of vision.
Contact
Ilaria Testi
The London Clinic Eye Centre
119 Harley Street
London W1G 6AU
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