Acute posterior multifocal placoid pigment epitheliopathy

Synonyms

6

Overview

Acute posterior multifocal placoid pigment epitheliopathy is an acquired, inflammatory eye condition affecting the retina, retinal pigment epithelium (pigmented layer of the retina), and choroid. It usually affects both eyes and is characterized by multiple, yellow-white lesions in the back of the eye. The condition can significantly impair visual acuity if the macula is involved. APMPPE typically resolves on its own in weeks to months. While the cause is unknown, about a third of cases appear to develop after a flu-like illness. Non-ocular symptoms are uncommon, but cerebral vasculitis can be present and may cause permanent and/or severe neurological complications.

It occurs more commonly in females, and is more likely to affect a persons between 20–30 years of ages but has been seen in people 16 to 40 years of age . It is known to occur after or concurrently with a systemic infection (but not always), showing that it is related generally to an altered immune system. Recurrent episodes can happen, but is extremely rare.

Symptoms

Prior to onset of the condition, about a third of people have flu-like or viral symptoms such as fever, swollen lymph glands, nausea, vomiting, joint pain and/or tenderness. Headaches may also be present. Rarely, there may be neurological signs such as temporary loss of speech (aphasia) and/or weakness of the arms and legs.

In the early stages of APMPPE, affected people may notice areas of visual blotchiness; flashes of light (photopsia) caused by irritation of the retina; distortion of shapes (metamorphopsia); increased sensitivity to light (photophobia); and/or conjunctivitis. Later, affected people usually develop impairment of vision. In rare cases, vision impairment may be severe.

In most cases, the disorder resolves within a few weeks without permanent loss of visual acuity. However, in some cases, visual acuity does not improve. Neurological symptoms develop in some affected people and should be evaluated and treated promptly, as neurological involvement can result in severe complications.

There are rare cases of chronic or recurrent APMPPE; it has been suggested that these cases represent relentless placoid chorioretinitis. This is a rare condition characterized by the development of multiple inflammatory lesions resembling those seen in APMPPE. However, unlike in APMPPE, the lesions continue to expand in size and number, with a relentless course over many months.

Causes

APMPPE has been known to occur after different conditions or in conjunction with a broad variety of disorders, as follows:

  • Tuberculosis exposure
  • Sarcoidosis
  • Mumps
  • Lyme disease
  • Nephritis
  • Hepatitis B vaccination
  • Group A streptococcal infection
  • Swine flu vaccination
  • Tuberculin skin testing
  • Penicillin therapy
  • Thyroiditis
  • Erythromycin therapy
  • Toxoplasmosis
  • Adenovirus 5 infection
  • Use of oral contraceptives
  • Use of hormone replacements
  • Clear cell renal cell carcinoma
  • Systemic necrotizing vasculitis
  • Ulcerative colitis
  • Meningococcal C conjugate vaccine
  • Adenovirus infection

Diagnosis

Diagnosis is made by an ophthalmologist during eye examination. Further tests such as Fluorescein angiography and/or lumbar puncture is usually performed to confirm the diagnosis. Similar auto immune disorder that can be confused with APMPPE, is neuro sarcoidosis.

Prognosis

 In many affected people, visual acuity returns to the range of 20/20 to 20/40. However, some people have permanent vision loss or long-term functional ocular symptoms. Other ocular and non-ocular symptoms usually resolve on their own and are not life-threatening. In affected people with cerebral vasculitis, there can be permanent and/or severe neurological complications; there have been instances of death following an episode of cerebral vasculitis.

These occurrences can start with mild headaches that steadily increase in pain and threshold and include attacks of dysesthesia. It has been found that this type of deterioration happens usually if the lesions involve the fovea.

Treatment

The treatment of APMPPE is somewhat controversial, but the general consensus is that no treatment seems to alter the course of the ocular lesions. In cases complicated by subretinal neovascularization (growth of new blood vessels), laser photocoagulation may be useful.

In most cases, the lesions resolve spontaneously and no therapy is required. Some clinicians have used corticosteroids (which suppress inflammation) to treat the ocular findings and any severe systemic involvement. However, there is no evidence that treatment with corticosteroids affects the visual outcome. The use of steroids has also been suggested when treating cases where the macula is involved. Cycloplegics may be useful for severe iritis, which is an uncommon finding.

It is recommended that people with questions about treatment options for themselves or family members speak with their health care provider.

Resources

  • NIH