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Vogt-Koyanagi-Harada Disease (VKH

Vogt-Koyanagi-Harada Disease (VKH)



Overview

Vogt-Koyanagi-Harada disease (VKH) is a rare condition that causes inflammation and damage throughout your body. It’s also known as Vogt-Koyanagi-Harada syndrome.

VKH disease can affect your:

Eyes

Inner ear

Skin

Hair

Brain

Spinal cord

Vogt-Koyanagi-Harada can permanently damage your vision and hearing if it’s not treated quickly. Visit a healthcare provider or eye doctor if you notice any sudden changes.

Symptoms

VKH causes lots of symptoms. You’ll notice some right away. It usually affects both your eyes first. Vision changes are the first sign. Symptoms will appear in other parts of your body later. There are four phases:

Prodromal phase

Acute uveitic phase

Chronic phase

Recurrent chronic phase

Prodromal phase

Prodrome is the medical name for an early sign or symptom. This first phase of VKH disease is short. It usually lasts around a week.

Symptoms in this phase often resemble a flu-like illness. They can include:

Eye pain, especially pain that feels like it’s coming from deep inside or behind your eyes

Dizziness or a feeling of vertigo

A ringing in your ears like tinnitus

Light sensitivity

Headaches

Fever

Symptoms that seem like meningitis, including fever

Meningitis is a medical emergency. Go to the emergency room if you think you might have it.

Acute uveitic phase

Acute means sudden. This phase happens immediately after the prodromal phase. Uveitis is inflammation inside your eye. In this phase, VKH disease causes inflammation in the choroid and retina inside your eyes. This phase usually lasts several weeks.

Symptoms can include:

Blurry vision

Severe light sensitivity

Red eye

Pain around or in your eye

Chronic phase

Chronic means long-lasting. This phase of Vogt-Koyanagi-Harada disease can last for weeks or months after the acute uveitic phase. You may still have symptoms of all the earlier stages. Symptoms will start to appear in other parts of your body. They can affect your hearing, skin and hair. You might notice:

Patches of your skin losing color or pigmentation, especially on your head, eyelids and belly

Patches of hair losing all color, especially in your eyebrows, eyelashes and scalp

Alopecia or significant hair loss

Worsening tinnitus

Hearing loss

Some healthcare providers call this the convalescent stage. Convalesce is the medical term for recovering or getting better. In this phase, symptoms may not develop as quickly. And you can recover from existing symptoms.

Recurrent chronic phase

This phase is the last to develop. Something that recurs goes away and comes back. In this case, the inflammation inside your eyes comes back. It might be more severe than before.

In this stage, you may not be able to feel changes inside your eye. But your eye doctor might see:

Increased inflammation

Small growths called granulomas forming

Leaking blood vessels

You might lose some of your vision. It may look like something is blocking part of your vision.

This stage is when you have the highest risk of complications, including:

Glaucoma

Cataracts

Neovascularization

Permanent hearing loss

Permanent low vision or blindness

Causes

Experts aren’t sure what causes Vogt-Koyanagi-Harada disease. Researchers are still studying why it happens. It may be an autoimmune disease. These happen when your immune system damages your body instead of protecting it.

A genetic variation that affects human leukocyte antigens (HLA) might cause VKH disease. HLA are proteins in your cells. Normally, they help your immune system tell the difference between healthy cells and those that shouldn’t be in your body. Experts think a genetic variation that affects your immune system’s ability to read HLA may cause it to attack healthy cells that it usually wouldn’t. That might lead to inflammation and other Vogt-Koyanagi-Harada syndrome symptoms.

Risk factors for Vogt-Koyanagi-Harada syndrome

Anyone can develop VKH disease. You’ll have a higher risk if you’re from certain ethnic backgrounds, including:

Asian

Indian

Native American

Hispanic

Middle Eastern

It usually affects people between the ages of 30 and 40. But it can develop at any time in your life.

Diagnosis

A healthcare provider will diagnose VKH by dilating your eyes and performing a full eye exam. Your provider will check your vision and look inside your eyes. They’ll check your hearing if it’s affected. Or they’ll refer you for a hearing test.

Tell your provider when you first noticed any symptoms or changes. Make sure to share how quickly the changes came on. That can be a clue, especially if your vision or hearing suddenly gets worse.

Because VKH disease is so rare, your provider will rule out other, more common conditions first. They’ll use a few tests to help cross possibilities off the list. You may need:

A CT scan

An MRI

Angiography

Optical coherence tomography (OCT)

A lumbar puncture

Blood tests

A hearing test

Types of Vogt-Koyanagi-Harada syndrome

Your provider might classify VKH into one of three types:

Complete: Complete VKH means you have symptoms that affect your eyes, skin, and your brain and nerves (your neurological system).

Incomplete: This is having VKH symptoms in your eyes and one of the other two places. So, you could have eye symptoms and neurological issues. Or eye symptoms and skin changes. But not all three.

Suspected: This is just what it sounds like. Your provider thinks you might have VKH, but they can’t diagnose it for sure yet. Usually, this means you have VKH-like inflammation inside your eyes, but no symptoms anywhere else.

Treatment

The goal of treating VKH is to prevent permanent damage to your vision, hearing and skin. Your healthcare provider will prescribe a combination of medications, including:

Corticosteroids: These are prescription anti-inflammatory medications. They’ll reduce inflammation inside your eyes and may prevent damage. You might need more than one type. Your provider will tell you what to expect.

Immunosuppressants: These are medications that hold back your immune system. They make it less active. This can help prevent your immune system from targeting healthy cells throughout your body.

You may need other treatments for specific symptoms as they happen. Your provider will let you know what’s best for you. Make sure you tell them if you notice any new symptoms.

Type of Doctor Department : An ophthalmologist

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