Harada's Disease

Eye Deceases

Harada's Disease / Vogt–Koyanagi–Harada Syndrome

Vogt-Koyanagi-Harada is a rare autoimmune disorder which involves multiple organs like eyes, ears and skin.  It is usually caused in middle aged adults.  It affects the melanocytes of skin, eyes and ears. Vogt-Koyanagi-Harada has several synonyms in medicine as Harada’s disease, Harada’s syndrome, VKH syndrome, Uveomeningitis syndrome. Harada’s disease is an acute diffused Choroiditis. Men and women are affected at same rate.

ICD-10 H20.8
ICD-9 364.24
DiseasesDB 13983
eMedicine derm/739
MeSH D014607

Harada's Disease Signs and Symptoms

The disease starts with the symptoms like headache. Many neurological symptoms are also associated with it such as severe headache, nausea, vertigo and giddiness.  Loss of hair and hearing loss are also some symptoms observed in Vogt-Koyanagi-Harada disease. Ocular symptoms consists of inflammation and bilateral. Uveitis is the characteristic of the Harada’s disease. The anterior uveitis shows symptoms such as photophobia, redness, conjunctivitis, iris nodules. Posterior symptoms are floating pigments, visual impairment, retinal edema, swelling in the optical nerve. Secondary symptoms include cataract and glaucoma.

Harada's Disease Treatment

Unilateral and bilateral ocular inflammatory diseases are usually treated by corticosteroids. Anterior and posterior ocular diseases are treated by administering steroid eye drops. Cyclopegic drops are used to relieve the symptomic pains in Iridocyclitis. Cyclotoxic agents are used sometimes in the curative procedure.

For bilateral inflammatory Harada’s disease and severe visual loss, therapy involves administration of systemic prednisone (1-2 mg/kg/d). Intravenous dose of methylprednisolone (up to 1 g/d) for many days is given to some patients observed with severe inflammation. Oral prednisone (1 mg/kg/d) is also given in form of tablets if needed.  Though, the steroids are given intravenously there is no significant alteration to the visual outcome or the ocular complications are not further hindered.

Many of the patients suffering from Harada’s disease require therapy for 6 months and sometimes up to 1 year. Systemic therapy should be continued for the next three months after the start of the disease to reduce the risk of recurrence.

 Some Harada’s disease patients show no recovery with the steroid therapy and hence an immunodulatory drug like Cyclosporine is given alone or with the combination of steroids.

 Topical therapy includes corticosteroids, such as prednisolone acetate for the treatment of anterior uveitis. In severe Harada’s disease, the drops are administered as one drop per hour and later tapered depending upon the response. In case of patients with mild uveitis in Harada’s disease, one drop is administered topically at the intervals of 4-6 hours.

Topical Cyclopegics are used to relieve the uneasiness of ciliary contraction and to avoid the development of posterior synechiae. Homatropine 5% is used 2-4 times daily in patients with Vogt-Koyanagi-Harada and stopped when symptoms are relieved.

Harada's Disease Causes

Causes of Harada’s disease are unknown. Autoimmune disorders can cause Harada’s disease.

Harada's Disease Types

Vogt, Koyanagi, and Harada studied several patients for period of 20 years having bilateral uveitis, exudative retinal detachments, neurologic abnormalities, and disorders of the skin. Though there were differences in the patients, the research represented a spectrum of disease. The disorder was hence called as Vogt-Koyanagi-Harada disease.

International Committee on Nomenclature recognized criteria for the diagnosis of Vogt-Koyanagi-Harada disease which made its diagnosis simpler. It differentiated the disease into three forms

  1. Complete Vogt-Koyanagi-Harada Disease
  2. Incomplete Vogt-Koyanagi-Harada Disease
  3. Probable Vogt-Koyanagi-Harada Disease