Vogt-Koyanagi-Harada Syndrome

Vogt-Koyanagi-Harada Syndrome

Vogt- Koyanagi -Harada syndrome is a multifactorial disease whose cause is not yet fully understood. However, it can be assumed that the autoimmune reaction against the melanocytes is mediated by the T cells. A virus as a trigger is also possible. The main symptom of the disease is chronic uveitis. Early diagnosis and early initiation of aggressive therapy are important to preserve vision.

What is Vogt-Koyanagi-Harada Syndrome?

Vogt-Koyanagi-Harada syndrome is a multifactorial disease in which chronic uveitis, a chronic inflammation of the iris, is the main feature. The main symptom, chronic uveitis, can be classified according to the anatomical site of origin. For hemolytic uremic syndrome (hus), please visit nonprofitdictionary.com.

There is a subdivision into: anterior uveitis, posterior uveitis, intermediate uveitis and panuveitis. In anterior uveitis, the front part of the iris is affected, and a further differentiation can be made between iritis and cyclitis. If uveitis is posterior, the posterior parts of the iris are affected – the retina, choridea, corpus vitreum, macula or otic nerve.

Intermediate uveitis, on the other hand, is an inflammation in the central area of ​​the iris. If panuveitis is present, all parts of the iris are affected by the inflammation. Symptoms of uveitis, common to all variants, include pain, sensitivity to light, tearing, and a foreign body sensation.

In anterior uveitis, there is also reddening of the eye, whereas in posterior uveitis, visual acuity is impaired at the center. Because of the symptoms, medical laypeople often confuse uveitis with conjunctivitis. In addition to the described chronic inflammation of the iris, various secondary diseases occur.

These include retinal detachment, vitreous opacity, papillitis with atrophy of the optic nerve and glaucoma, meningitis, various skin symptoms such as poliosis (whitening of body hair), vitiligo (disease characterized by loss of pigment) and hair loss (alopecia).

Inner ear symptoms can also sometimes occur, such as increased sensitivity to high-pitched sounds. The disease occurs primarily in heavily pigmented people between the ages of 20 and 50.

Causes

The exact causes of the syndrome have not yet been clarified. However, it can be assumed that the disease is triggered either by a virus or by a T-cell-mediated autoimmune reaction against the melanocytes. The T cells belong to the group of lymphocytes.

They are formed like other blood cells in the bone marrow. Differentiation takes place in the thymus. Because of the location of differentiation, the cells are called T cells. (T stands for thymus) The T cells recognize foreign antigens via a receptor, the so-called T cell receptor (TCR).

So they represent part of the human immune system and react to foreign antigens. Melanocytes are pigment-forming cells in the skin that make up about five to ten percent of the cells in the epidermal basal cell layer. In addition to the epidermal basal cell layer, the melanocytes can be found in the outer root sheath and in the bulb of the hair follicle.

The melanocytes have the melanosome, a cell organelle which arises from unpigmented premelanosomes, is structureless, heavily pigmented and is an electron microscopic characteristic of the melanocyte. The skin pigment melanin, which is synthesized from tyrosine, is stored in the melanosome and released to neighboring migrating keratinocytes. The melanocytes themselves are not heavily pigmented because they constantly pass the melanin to the keratinocytes.

The more melanin the human body produces, the more pigmented it is. If the T cells now react to the melanocytes, this can be described as an autoimmune reaction, since the T cells, contrary to their actual function, do not react to a foreign antigen but to the body’s own cells. In this case, one speaks of so-called autoimmune diseases – the immune system or the immune defense is directed against the body’s own structures.

Symptoms, Ailments & Signs

In the clinic, the Vogt-Koyanagi-Harada syndrome is particularly noticeable due to the chronic uveitis and various secondary diseases. Many patients confuse (chronic) uveitis with conjunctivitis because the symptoms are sometimes very similar. Pain, a foreign body sensation, reddening and sensitivity to light occur in anterior uveitis.

In the posterior variant of uveitis, on the other hand, there is no reddening of the eye, but a reduction in visual acuity. In addition to the chronic inflammation of the iris, a variety of other ophthalmological diseases occur. Detachment of the retina, opacity of the vitreous body and papilitis with secondary atrophy of the optic nerve and glaucoma should be mentioned here.

In addition to the diseases of the eyes mentioned, there are many other secondary diseases: meningitis, skin and inner ear symptoms. People who are more heavily pigmented are particularly affected. The onset of the disease usually falls between the ages of 20 and 50.

Diagnosis & course of disease

The diagnosis is made clinically. This means that there is no separate test procedure to diagnose Vogt-Koyanagi-Harada syndrome. The syndrome is diagnosed solely on the basis of the existing symptoms or the existing secondary diseases.

Based on the existing symptoms outside of the ophthalmological complaints, a complete, an incomplete or a probable diagnosis is spoken of. The prognosis of the disease with regard to the preservation of vision is good if the syndrome is detected in good time and treatment is started in good time.

Complications

The Vogt-Koyanagi-Harada syndrome can lead to a number of different symptoms and thus have a very negative effect on the quality of life of those affected. Most patients often suffer from conjunctivitis, which in the worst case can lead to blindness. Pain or a foreign body sensation can also occur in the eye and have a negative effect on the eyesight of the person concerned.

Furthermore, the Vogt-Koyanagi-Harada syndrome leads to a significantly increased sensitivity to light. The iris can also become inflamed and the patient’s vision can continue to deteriorate. Sudden visual problems can lead to severe psychological problems or depression, especially in children or young people.

In the case of blindness, those affected are dependent on the help of other people or their parents in their lives and are significantly restricted in their everyday life. Since the Vogt-Koyanagi-Harada syndrome cannot be treated causally, only the individual symptoms are treated with medication. As a rule, there are no complications or other complaints.

However, the patients are always dependent on taking the medication. Vogt-Koyanagi-Harada syndrome does not negatively affect the patient’s life expectancy.

When should you go to the doctor?

In the case of Vogt-Koyanagi-Harada syndrome, a visit to a doctor is usually always necessary. This disease cannot heal on its own, so the person affected should contact a doctor as soon as the first symptoms and signs of the disease appear. Early diagnosis with subsequent treatment always has a very positive effect on the further course of the disease and can prevent further complications.

A doctor should be contacted with Vogt-Koyanagi-Harada syndrome if the patient suffers from very severe pain in the eyes. This pain occurs for no particular reason and will not go away on its own. Furthermore, a foreign body sensation in the eye can indicate the syndrome and should also be examined by a doctor.

The visual acuity also decreases and there is severe reddening of the eye. In some cases, a clouded vitreous can also indicate Vogt-Koyanagi-Harada syndrome. As a rule, the disease can be easily recognized and treated by an ophthalmologist. The further course depends heavily on the time of diagnosis.

Treatment & Therapy

Therapy is aggressive with high-dose steroids. In the longer term, immunosuppressants, substances that suppress the body’s own immune system, can also be used. Ciclosporin, for example, could be mentioned as an active ingredient here. The steroids are given systemically. The steroids have an anti-inflammatory effect.

Prevention

Prevention is not possible.

Aftercare

Those affected with Vogt-Koyanagi-Harada syndrome usually only have very limited and very few measures of direct aftercare available. Since this is a congenital disease, it usually cannot be completely cured. Therefore, ideally, the sufferer should see a doctor at the first signs and symptoms of the condition and also initiate treatment to prevent the onset of other symptoms.

Genetic testing and counseling is also very useful if you wish to have children and should be carried out in order to prevent the syndrome from occurring in the offspring. Most of those affected are dependent on various surgical interventions with which the symptoms and deformities can usually be alleviated well.

The person concerned should definitely rest and rest after the operation. Efforts or stressful and physical activities should be avoided in order not to unnecessarily burden the body. The treatment of the Vogt-Koyanagi-Harada syndrome continues to be carried out by means of physiotherapy or physiotherapy. Those affected can also repeat many of the exercises at home and thus accelerate healing. In some cases, this disease also limits the life expectancy of the sufferer.

You can do that yourself

Vogt-Koyanagi-Harada syndrome requires medical attention. The therapy of malformations and functional disorders of the body can be supported by various self-help measures.

The skin symptoms can be treated with natural care products from the drugstore or pharmacy. The use of aggressive creams or ointments should first be discussed with the doctor, as complications can occur. If there is increased sensitivity to high-pitched sounds, which is part of the typical inner ear symptoms, ear protection can be worn. In addition to earplugs, classic earmuffs are also available. In the case of severe symptoms such as fever or gastrointestinal complaints, such as those that occur in connection with meningitis, rest and rest are the order of the day. In addition, you should drink plenty of water. It is important to inform the doctor about serious complications. In the case of severe symptoms, the medical emergency service is the right contact person.

In the case of papillitis, the affected eye must be rested. Suitable eye drops from the pharmacy relieve the symptoms. In addition, the affected eye should be protected from direct sunlight and other irritants. Which measures are useful and necessary for the Vogt-Koyanagi-Harada syndrome is best discussed with the responsible doctors.

Vogt-Koyanagi-Harada Syndrome