Vogt-Koyanagi-Harada Syndrome - Causes, Symptoms, Diagnosis, and Treatment

Creator: Dr. Namrata Singhal
Published: 2023-04-05T18:43:01

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Vogt-Koyanagi-Harada syndrome is a brain disorder affecting vision and hearing in particular. Read this article to know in detail about the disease.

Written by

Dr. Namrata Singhal

Medically reviewed by

Dr. Abhishek Juneja

April 5, 2023

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Contents

What Is the Etiology of Vogt-Koyanagi-Harada Syndrome?
What Is the Epidemiology of Vogt-Koyanagi-Harada Syndrome?
What Are the Signs and Symptoms of Vogt-Koyanagi-Harada Syndrome?
How Is the Diagnosis of Vogt-Koyanagi-Harada Syndrome Made?
What Is the Treatment of Vogt-Koyanagi-Harada Syndrome?
What Are the Complications of Vogt-Koyanagi-Harada Syndrome?

Introduction

Vogt-Koyanagi-Harada syndromeis a disorder of the central nervous system affecting the vision and hearing abilities of an individual in particular. The syndrome is initially characterized by severe headaches, intense pain in the eyes, vertigo (dizziness), and feeling of nausea.

Vogt-Koyanagi-Harada disease is an uncommon disorder. It is of unknown cause that involves multiple organ systems, including the eyes, auditory system (ears), skin, and the meninges (the protective covering of the brain and spinal cord). Rapid vision loss is the first symptom to occur. Severe pain in the head, spinning head (vertigo), vomiting and nausea, and feeling drowsy are some of the other neurological signs manifested. Hearing loss, alopecia (hair loss), and skin pigmentation may occur along with loss of color from the hair and eyelashes (poliosis).

Synonyms of Vogt-Koyanagi-Harada Syndrome:

Alopecia-poliosis-uveitis-vitiligo-deafness-cutaneous syndrome.
Harada syndrome.
Uveo Meningitis disease.
VKH syndrome.

What Is the Etiology of Vogt-Koyanagi-Harada Syndrome?

The exact cause of the disease is not known fully but it is postulated that patients often develop a T-cell-mediated autoimmune response against the melanocytes (melanin-producing cells) after being exposed to a viral infection. It is believed to be an autoimmune disorder that occurs when the natural defenses of the body's immune system such as antibodies, and lymphocytes, begin to act against the healthy tissues in the body.

Viruses like cytomegalovirus (CMV) and Epstein-Barr virus have been found to play an important part in the pathogenesis of the disease. Vogt- Koyanagi-Harada disease is thought to occur because of the lack of tolerance in the melanocytes. This leads to a granulomatous inflammation of the non-necrotizing type in the eyes, ears, skin, and hair.

Certain genetic markers found on chromosome 6 are also believed to pose risk for the development of Vogt-Koyanagi-Harada syndrome. Some scientists consider a genetic predisposition for the disease since it has developed in a set of twins.

What Is the Epidemiology of Vogt-Koyanagi-Harada Syndrome?

It is a very uncommon disorder affecting both men and women equally. It is found that the disease is more commonly presented in patients suffering from uveitis. In fact, it is the most common causative factor of uveitis in Indians followed by Thailand. The disease is more prevalent in Asian and American people than in European people. The disease can occur at any age though it most commonly occurs at the age of 30 to 40 years.

What Are the Signs and Symptoms of Vogt-Koyanagi-Harada Syndrome?

The disease is characterized by severe headache along with severe pain in the eyes, followed by vertigo (spinning head), and vomiting in the prodromal phase. Inflammation of the eyes (uveitis) along with progressive loss of vision is the other symptoms that follow the prodromal symptoms in the acute uveitis phase in a few weeks. Either one or both eyes are affected by the disease. Loss of hearing sensation and retinal detachment also occurs in another few weeks.

The chronic phase follows the acute phase in a few weeks. The chronic stage is characterized by visible eye and skin changes. Loss of color in the choroid layer of the eye along with the presence of yellowish nodules developed in some parts of the retina. Changes in the skin are accompanied by the appearance of a condition known as vitiligo (loss of skin pigmentation) that leads to the formation of white patches on the skin. The chronic stage usually lasts from several weeks to several months to several years.

The chronic phase might reoccur in the recurrent phase characterized by panuveitis (all the layers of the eye are inflamed).

How Is the Diagnosis of Vogt-Koyanagi-Harada Syndrome Made?

There is a diagnostic criterion for the disease that includes inflammation of the eyes bilaterally, with no other evidence present of another eye disorder causing this inflammation, and the patient has no history of injury to the eyes or eye surgery. Three categories of the disease have been established for each diagnosis:

Complete Vogt-Koyanagi-Harada Syndrome: Bilateral panuveitis involving retinal detachments usually develop in patients. The complete form of this disorder includes clinical signs such as tinnitus, neck rigidity, skin changes like white patches on the arms, sudden alopecia, and even loss of color in the hairs, and eyelashes.
Incomplete Vogt-Koyanagi-Harada Syndrome: The changes in the eye are similar to those of the patients having a complete form of the disease but these patients will exhibit either neural or dermatological signs.
Probable Vogt-Koyanagi-Harada Syndrome: The eye changes are similar to the patients exhibiting a complete form of the disease but without showing any neurologic and dermatological signs.

The testing used by an eye specialist or neurologist in order to determine the presence of the disease includes a spinal tap (lumbar puncture), the injection of dye in the blood vessels to scan them (angiography), and ultrasonography.

What Is the Treatment of Vogt-Koyanagi-Harada Syndrome?

The gold standard treatment of Vogt-Koyanagi-Harada syndrome is the intake of initial high-dose systemic corticosteroids followed by immunosuppressive drug therapy. Another treatment modality is symptomatic and supportive to halt the process of inflammation.

The key to successful treatment is the aggressive use of systemic glucocorticoids. Intravitreal injections are also planned to treat eye inflammation.

What Are the Complications of Vogt-Koyanagi-Harada Syndrome?

Vogt-Koyanagi-Harada Syndrome is a systemic autoimmune inflammatory disease which if left untreated can lead to severe complications of the disorder including loss of hearing, dysacusis (hearing impairment), tinnitus (ringing bell in the ears), aural fullness (sensation of ear blockage), vitiligo (white patches appearing on the skin), vertigo (dizziness), and ophthalmic (eye) complications.

Conclusion

It is crucial to make the patients understand that Vogt-Koyanagi-Harada syndrome is an autoimmune systemic disorder that can recur and even relapse. If the disease is left untreated, the disease recurs repetitively and might lead to several complications, and patients often need immunosuppressive therapy for lifelong support. The healthcare professional should educate the patients on the various aspects and clinical manifestations of the syndrome to identify the recurrent form of the disease and seek medical help immediately. Early diagnosis along with both steroidal treatment and anti-steroidal immunosuppressants is a crucial requirement for the management of this syndrome.

Frequently Asked Questions

1. What Are the Potential Differential Diagnoses for Vogt-Koyanagi-Harada Syndrome?

Other uveitis disorders, such as sympathetic ophthalmia, Behçet's disease, and posterior scleritis, are included in the Vogt-Koyanagi-Harada (VKH) syndrome differential diagnosis. Due to its distinctive clinical characteristics, such as bilateral panuveitis, serous retinal detachments, cutaneous and auditory symptoms, it is essential to distinguish VKH from similar illnesses.

2. How Does Vogt-Koyanagi-Harada Syndrome Differ from Sympathetic Ophthalmia?

Both sympathetic ophthalmia and Vogt-Koyanagi-Harada syndrome are autoimmune diseases that affect the eyes. But their triggers are different. While sympathetic ophthalmia develops as an immunological reaction to ocular damage or surgery in one eye, affecting the other eye, VKH is often brought on by a viral or autoimmune cause.

3. What Factors Increase the Risk of Secondary Glaucoma in Individuals with Vogt-Koyanagi-Harada Disease?

If persistent eye inflammation caused by Vogt-Koyanagi-Harada (VKH) illness is not appropriately treated or controlled, secondary glaucoma may develop. This happens as a result of the buildup of inflammatory cells and fluid inside the eye, which raises intraocular pressure (IOP). The optic nerve, which carries visual information from the eye to the brain, might gradually suffer damage from increased IOP. Inadequate treatment and delayed diagnosis of VKH might aggravate this process, increasing the risk of subsequent glaucoma in patients. Early identification and rapid, efficient care of VKH are essential to reducing this risk and maintaining both eyesight and the health of the optic nerve.

4. What Is the Characteristic Feature of Vogt-Koyanagi-Harada Syndrome?

Bilateral, widespread, granulomatous panuveitis, which involves inflammation of the uvea, the main layer of the eye, is a defining feature of the Vogt-Koyanagi-Harada (VKH) syndrome. In addition, VKH frequently displays symptoms on the skin and in the ears in addition to accompanying manifestations in the eyes. Prodromal, uveitic, convalescent, and chronic recurring are the usual stages that the illness develops through. The successful management of VKH and the avoidance of long-term consequences depend on the accurate diagnosis and prompt intervention throughout these stages, which are characterized by a variety of clinical characteristics.

5. Can the Optic Nerve be Surgically Repaired?

Optic nerve repair is a difficult and complicated medical procedure. The optic nerve's low potential for regeneration, in contrast to several other tissues in the body, makes it challenging to entirely repair if injured. Although surgical procedures like optic nerve decompression and optic nerve sheath fenestration are available, their effectiveness is largely dependent on the severity and kind of the optic nerve lesion. Despite breakthroughs in medical research, it is still difficult to fully restore optic nerve function, and results might vary from person to person.

6. What Are the Differential Diagnoses for Campomelic Dysplasia?

Other skeletal dysplasias, such as thanatophoric dysplasia, achondroplasia, and certain genetic conditions that impact bone formation, are differential diagnoses for campomelic dysplasia. Due to the overlap in clinical characteristics between these illnesses and bone abnormalities, it is crucial for medical personnel to distinguish between them using precise diagnostic criteria and, if required, genetic testing.

7. Can Cervical Dysplasia Resolve on Its Own?

When cervical dysplasia is low-grade, it occasionally resolves spontaneously. However, in order to precisely monitor the changes and, if required, treat them, high-grade dysplasia frequently necessitates medical intervention, such as colposcopy and biopsy. To manage cervical dysplasia and stop it from progressing to more serious problems, such cervical cancer, regular follow-up visits with a healthcare professional are crucial.

8. What Is Genetic Dysplasia?

A set of genetic illnesses known as genetic dysplasia are characterized by aberrant tissue or organ growth or development as a result of abnormalities in certain genes essential for proper development. Depending on the genes involved and the period of development at which the mutations arise, these mutations can cause a wide range of morphological deformities or functional impairments by interfering with the normal processes of tissue creation.

9. Is There a Hereditary Component to Dysplasia?

Dysplasia can run in families. However, the kind of dysplasia and the underlying genetic abnormalities determine whether or not it is hereditary. While certain types of dysplasia may be random and without a definite family history, others may be brought on by inherited genetic abnormalities. Different dysplasias can have different inheritance patterns; some may be autosomal dominant or recessive, while others may entail X-linked inheritance. To comprehend the precise genetic components and inheritance pattern linked to a certain dysplasia, it is crucial to speak with a genetic counselor.

10. What Distinguishes a Disease From a Syndrome or a Disorder?

A syndrome is a group of symptoms and indications that frequently occur together but may not all have a single underlying cause, whereas the term "disease" is used more broadly to refer to a pathological condition that affects the body. Any aberrant situation, including illnesses, syndromes, and other anomalies of the health, is referred to as a disorder in general.

11. How Is Campomelic Dysplasia Inherited?

Campomelic dysplasia is usually inherited in an autosomal dominant manner. This means that if a person carries a mutation in the responsible gene, they have a 50% chance of passing the condition on to their offspring. The autosomal dominant inheritance pattern means that the presence of one copy of the mutated gene is sufficient to cause the disorder, and it is equally likely to be passed from either parent.

12. Is Dysplasia Generally an Inherited Condition?

Dysplasia can be hereditary in some cases, but it's not a universal trait across all forms of dysplasia. The inheritance pattern depends on the specific type of dysplasia and the genetic mutations involved. Some dysplasias are caused by spontaneous genetic mutations and are not inherited, while others can be passed down from one generation to the next through autosomal dominant or recessive inheritance patterns, or via X-linked inheritance, depending on the genetic basis of the condition.

13. What Is the Most Common Lethal Form of Dysplasia?

One of the most prevalent deadly types of skeletal dysplasia is called thanatophoric dysplasia. It is a severe hereditary condition that causes aberrant bone growth, which results in short limbs, an abnormally tiny ribcage, and other skeletal anomalies. Due to significant respiratory and neurological difficulties, infants born with this disease frequently have an extremely short life expectancy.

14. What Neurological Conditions Can Lead to Vision Problems?

Various brain issues, such as strokes, brain tumors, multiple sclerosis, or head injuries, can result in vision problems by disrupting the normal functioning of the visual pathways or the regions of the brain responsible for processing visual information. These conditions can lead to vision impairment or abnormalities due to damage to the optic nerves, visual cortex, or other critical brain structures involved in vision processing.

15. Which Specific Part of the Brain is Responsible for Processing Sight and Hearing?

Different parts of the brain are principally responsible for processing hearing and vision. The rear of the brain's occipital lobes, where visual information from the eyes is interpreted, is where sight is mostly processed. In contrast, the auditory cortex in the temporal lobes, where sound impulses are heard and interpreted, is where hearing is predominantly processed. We are able to separately process visual and aural inputs thanks to these different brain areas.

Sources

Dr. Abhishek Juneja

Neurology

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