What are the other Names for this Condition? (Also known as/Synonyms)

• ARPE (Acute Retinal Pigment Epitheliitis)
• Krill's Disease

What is Acute Retinal Pigment Epitheliitis? (Definition/Background Information)

• Acute Retinal Pigment Epitheliitis (ARPE) is an uncommon retinal (macular) condition of unknown cause that affects young adults who are healthy
• ARPE is a self-limited condition that resolve without treatment. It may present symptoms, such as blurred vision, blind spots, and retinal inflammation

Who gets Acute Retinal Pigment Epitheliitis? (Age and Sex Distribution)

• Individuals of any age category may be affected; however, a majority of the cases are reported in young and middle-aged adults in the 20-50 years age group
• Both males and females are equally affected
• Worldwide, individuals of all races and ethnicity are prone to developing the condition

What are the Risk Factors for Acute Retinal Pigment Epitheliitis? (Predisposing Factors)

• The risk factors for Acute Retinal Pigment Epitheliitis are unknown

It is important to note that having a risk factor does not mean that one will get the condition. A risk factor increases one’s chances of getting a condition compared to an individual without the risk factors. Some risk factors are more important than others.

Also, not having a risk factor does not mean that an individual will not get the condition. It is always important to discuss the effect of risk factors with your healthcare provider.

What are the Causes of Acute Retinal Pigment Epitheliitis? (Etiology)

The cause of Acute Retinal Pigment Epitheliitis is not clearly understood.

• It is reported that a viral infection with flu-like symptoms may precede the condition
• However, the correlation between the two conditions is not known, and not all cases of ARPE show a preceding flu-like infection

What are the Signs and Symptoms of Acute Retinal Pigment Epitheliitis?

The signs and symptoms of Acute Retinal Pigment Epitheliitis may differ from one individual to another. It may be mild or severe, depending on several factors. These may include:

• In most cases, an acute/sudden onset of the condition is noted
• Central blind spot (scotoma) is observed
• Blurred vision
• Visual acuity (clarity of vision) may be slightly decreased
• Typically, only retinal inflammation is seen, and no other eye structures present inflammation
• The presence of several blind spots may affect partial vision (depending on their location)
• On funduscopic exams, retinal lesions may be observed

How is Acute Retinal Pigment Epitheliitis Diagnosed?

Acute Retinal Pigment Epitheliitis may be diagnosed by the following observations and tests:

• Physical examination and medical history evaluation (including history of any recent illness or other conditions)
• Assessment of signs and symptoms
• General eye exam
• Fundoscopic (ophthalmoscopic) examination by an eye specialist, who examines the back part of the eye (or the fundus)
• Visual acuity test using a special and standardized test chart (Snellen chart)
• Slit-lamp examination
• Visual evoked potential (VEP) test
• Fundus fluorescein angiography
• Optical coherence tomography (OCT) of eye

A differential diagnosis to eliminate other conditions may be considered, before arriving at a definitive diagnosis. These may include:

• Acute idiopathic maculopathy
• Acute macular neuroretinopathy
• Multiple evanescent white dot syndrome (MEWDS)
• Solar retinopathy

“Ophthalmoscopy discloses discrete pigment clumps with surrounding hypopigmented halos in the center of the macula.”

Reference: Chittum ME, Kalina RE. Acute retinal pigment epitheliitis. Ophthalmology. 1987 Sep;94(9):1114-9. doi: 10.1016/s0161-6420(87)33316-0. PMID: 3684229.

Many clinical conditions may have similar signs and symptoms. Your healthcare provider may perform additional tests to rule out other clinical conditions to arrive at a definitive diagnosis.

What are the possible Complications of Acute Retinal Pigment Epitheliitis?

The complications of Acute Retinal Pigment Epitheliitis may include:

• Emotional stress
• Partial loss of vision
• Decreased quality of life

How is Acute Retinal Pigment Epitheliitis Treated?

Presently, there are no treatment measures for Acute Retinal Pigment Epitheliitis.

• A wait and watch approach may be considered by the healthcare provider for several weeks to months
• Serial visual tests are considered important to record progress of the condition (if any)

How can Acute Retinal Pigment Epitheliitis be Prevented?

• Presently, it is difficult to prevent Acute Retinal Pigment Epitheliitis
• Undertake early and adequate treatment of any health issues, such as infections, is important

What is the Prognosis of Acute Retinal Pigment Epitheliitis? (Outcomes/Resolutions)

The prognosis of Acute Retinal Pigment Epitheliitis is typically good, since it is a self-limited condition and tends to resolve within 6-12 weeks.

• According to literature, around 90% of the individuals regain their normal sight within 8 weeks; however, changes to the retinal pigment epithelium (RPE) are mostly irreversible
• An early and appropriate diagnosis of the condition can help avoid unnecessary treatments
• Unfavorable prognosis may be noted in those with poor eyesight at onset of the condition and extensive retinal involvement

Additional and Relevant Useful Information for Acute Retinal Pigment Epitheliitis:

Please visit our Eye & Vision Health Center for more physician-approved health information:

https://www.dovemed.com/health-topics/vision-center/