Vogt-Koyanagi-Harada Disease - Symptoms, Diagnosis, and Treatment

Introduction

Vogt-Koyanagi-Harada disease is a rare disorder of unknown etiology that affects many body parts, such as the eyes, ears, skin, brain, and spinal cord. The most common symptom is the rapid loss of vision. Neurological symptoms are also associated with a syndrome, such as headaches, vertigo (dizziness), nausea, and drowsiness. In addition, loss of hair (alopecia) and loss of skin, head, and eyelash color is also seen in this disease. The exact cause is unclear, but this disease can occur because of an abnormal response of an immune system to viral infection, or genetic factors can also be involved.

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

• Headache.

• Pain in the eyes.

• Inflammation of the eyes (uveitis).

• The blurring of vision.

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

• The exact cause is unknown, but patients develop T cell-mediated immunity (destruction of infected cells by cytotoxic T cells) against melanocytes (cells in the skin that produce melanin) after recovery from viral infection.

• Cytomegalovirus and Epstein Barr virus can predispose to this disease.

• Recent studies have also shown the presence of HLA antigen (human leukocyte antigen that plays a significant role in disease and defense) associated with the disease.

What Are the Phases of Vogt-Koyanagi-Harada Disease?

There are four phases of Vogt-Koyanagi-Harada disease.

1. Prodromal Phase: The prodromal phase is self-limited and lasts for some hours and weeks. Patients show prodromal symptoms such as dizziness, eye pain, increased sensitivity to light, headache, meningeal signs, or fever.

2. Acute Uveitis Phase: Blurring of the vision and increased sensitivity to light is seen secondary to the choroid and retinal inflammation. Edema (fluid retention) can then extend to the retina, the optic nerve, or the ciliary body and thus can lead to retinal detachments.

3. Chronic Uveitis Phase: Chronic phase develops progressively, and there are other than ocular symptoms seen, such as hearing problems like tinnitus (ringing sound in the ears), dysacusis (distortion in frequency and intensity of sound), and also hearing loss, skin depigmentation, and ciliary depigmentation - commonly noted at the eyebrows, eyelashes, and scalp.

4. Recurrent Chronic Phase: The recurrent chronic phase consists of symptoms of panuveitis (inflammation of all layers of the uvea of eyes) and signs such as sunset glow fundus (fundoscopic finding characterized by orange-red discoloration), choroidal neovascular membranes (due to abnormal growth of blood vessels), neovascularization of the iris (growth of new blood vessels around the eyes), open and closed angle glaucoma (damages optic nerve and leads to vision loss or blindness) and subretinal fibrosis (wound healing response).

What Is the Diagnosis of Vogt-Koyanagi-Harada Disease?

• Fundus Fluorescein Angiography (FFA) - FFA is a test to identify the retina and choroid blood flow. FFA shows initially irregular focal or patchy fluorescence of the choroidal circulation. As the disease progresses, hyperfluorescent spots increase in size. Moth-eaten appearance is the hallmark of Vogt-Koyanagi-Harada disease.

• Indocyanine Green Angiography (ICG) - ICG is a diagnostic procedure used to examine choroidal blood flow and associated pathology. ICG shows hyper fluorescence and hyperfluorescent dark spots during the early phase of the disease. During the active stage, hyperfluorescent spots fade and are replaced by hyperfluorescent ones, and in the chronic stage, the hyperfluorescent dark spots are seen.

• Ultrasonography - Ultrasonography is done to evaluate the acute stages of the disease.

• Electroretinography - Electroretinography is done at the chronic stage of the disease.

• Laboratory Tests - Spinal fluid examination is done to diagnose the disease, and protein level is elevated in the early stages.

• Optical Coherence Tomography (OCT) - OCT is also done to evaluate the disease and will help evaluate the presence of subretinal fluid.

• Fundus Autofluorescence (FAF) - FAF is done to analyze the condition of the disease. In the active stage, FAF will show hyper autofluorescence and hypo autofluorescence. In addition, different patterns can be seen during the chronic stage, such as decreased autofluorescence, increased autofluorescence, and normal autofluorescence.

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

• Early diagnosis is essential to managing the disease.

• Aggressive treatment with Corticosteroids is undertaken to treat the disease.

• Ophthalmological and neurological assessment is mandatory in the treatment of Vogt-Koyanagi-Harada disease.

• Some theories state that using immunosuppressants should be the first line of treatment. The reason behind using immunosuppressants is that steroids have a lot of side effects. Azathioprine, Cyclosporine A, Mycophenolate Mofetil, and Rituximab have also been used in managing the disease.

• For the treatment of glaucoma, surgery is undertaken.

Surgery Includes:

1. Laser Iridotomy - A small hole is placed in the iris to drain the fluid from the hole with the help of a laser.

2. Surgical Iridectomy - Removal of part of the iris is done surgically.

3. Trabeculectomy - A flap is created in the sclera, and through the flap, fluid is drained that helps in lowering the pressure and helps in the treatment of glaucoma.

What Is the Differential Diagnosis of Vogt-Koyanagi-Harada Disease?

• Sympathetic ophthalmia (inflammation of the uveal tract).

• Intraocular lymphoma (a rare malignancy that can extend from the central nervous system and metastasize to the eyes).

• Bilateral diffuse uveal melanocytic proliferation (causes progressive visual loss and malignancy).

• Metastatic carcinoma (cancer cells break from the tumor, enter the bloodstream or lymph, and include other organs).

• Idiopathic uveal effusion syndrome (rare syndrome seen in males associated with increased sclera thickness).

• Benign reactive lymphoid hyperplasia (benign lesion involving conjunctiva).

• Sarcoidosis (growth of inflammatory cells called granulomas in lungs and lymph nodes).

• Vitiligo with poliosis (a disease of pigment cells of eyelashes, eyebrows, and skin).

• Scleritis (inflammation of scleral tissues).

• Peripheral anterior synechiae (adhesions are formed in the eye because of inflammation).

• Tuberculosis (bacterial infection caused by mycobacterium tuberculosis mainly affecting lungs and lymph nodes).

• Anterior granulomatous uveitis (a most common cause of inflammation and redness in the eyes).

• White dot syndromes (white lesions are seen in the retina and the choroid).

• Alezzandrini syndrome (rare syndrome causing blindness in patients).

• Alopecia areata (an autoimmune disorder that results in patchy hair loss).

• Piebaldism (absence of pigment cells called melanocytes).

• Syphilis (bacterial disease spreads through sexual contact).

Conclusion

Vogt-Koyanagi-Harada disease is a rare disorder characterized by severe inflammation in the eyes. This disease is seen in pigmented races around the fourth and fifth decades of life. It is seen more in women as compared to men. Early diagnosis, slow tapering of steroids, and immunosuppressants can help treat the disease.