Which are the symptoms of Vogt-Koyanagi-Harada Disease?

Vogt-Koyanagi-Harada (VKH) disease is a rare autoimmune disorder that primarily affects the eyes, skin, and other organs. It is characterized by a chronic inflammation of various tissues, resulting in a range of symptoms. The exact cause of VKH disease is not fully understood, but it is believed to involve an abnormal immune response triggered by genetic and environmental factors.

Ocular Symptoms:

One of the hallmark features of VKH disease is bilateral uveitis, which refers to inflammation of the uvea, the middle layer of the eye. This inflammation can affect multiple structures within the eye, leading to various visual disturbances. Common ocular symptoms include:

• Blurred vision: Patients may experience a decrease in visual acuity or have difficulty focusing.


• Photophobia: Sensitivity to light is a common symptom, causing discomfort in bright environments.


• Eye pain: Inflammation can result in eye discomfort or aching.


• Redness: The eyes may appear red or bloodshot due to the inflammation.


• Tearing: Excessive tearing or watery eyes can occur.


• Floaters: Dark spots or specks may be perceived in the visual field.


• Loss of vision: In severe cases, VKH disease can lead to partial or complete vision loss.

Systemic Symptoms:

VKH disease can also affect other parts of the body, leading to systemic symptoms that may precede or accompany ocular manifestations. These systemic symptoms can vary in severity and may include:

• Skin changes: Skin involvement can manifest as vitiligo (loss of skin pigmentation), hair whitening, or rashes.


• Hearing problems: Patients may experience tinnitus (ringing in the ears) or hearing loss.


• Neurological symptoms: Headaches, meningismus (stiff neck), or neurological deficits can occur.


• Generalized symptoms: Fatigue, fever, joint pain, and muscle aches may be present.


• Respiratory symptoms: Cough, shortness of breath, or respiratory distress can rarely occur.

Chronic and Recurrent Course:

VKH disease typically follows a chronic and recurrent course, with distinct phases of progression and remission. The disease can be divided into four stages:

1. Prodromal stage: This initial stage involves non-specific symptoms such as fever, headache, and flu-like symptoms.


2. Acute uveitic stage: Ocular symptoms become prominent during this stage, including uveitis, vision changes, and eye pain.


3. Convalescent stage: Inflammation begins to subside, and symptoms gradually improve. However, skin and auditory manifestations may persist.


4. Chronic recurrent stage: Recurrences of ocular and systemic symptoms can occur over months or years, requiring ongoing management and monitoring.

Diagnosis and Treatment:

Diagnosing VKH disease involves a combination of clinical evaluation, medical history, and specialized tests. These may include a comprehensive eye examination, imaging studies, blood tests, and analysis of cerebrospinal fluid. Early diagnosis is crucial to prevent complications and preserve vision.

Treatment of VKH disease aims to control inflammation, manage symptoms, and prevent recurrences. It typically involves a combination of corticosteroids and immunosuppressive medications to suppress the abnormal immune response. Close monitoring by an ophthalmologist and other specialists is essential to adjust treatment as needed.

In conclusion, Vogt-Koyanagi-Harada disease is a rare autoimmune disorder characterized by chronic inflammation of the eyes, skin, and other organs. The disease presents with ocular symptoms such as blurred vision, eye pain, and redness, as well as systemic manifestations including skin changes, hearing problems, and neurological symptoms. VKH disease follows a chronic and recurrent course, with distinct stages of progression and remission. Early diagnosis and appropriate treatment are crucial to manage symptoms, prevent complications, and preserve vision.