IN THE NAME OF GOD

Vogt-koyanagi-harada Ghanbari MD 1390:07:21

VKH syndrome Vogt-koyanagi syndrome - vogt (1906) & koyanagi (1929) - bilateral anterior uveitis, vitiligo, poliosis, and dysacusia. Harada disease - harada (1926) - posterior uveitis, CSF pleocytosis , exudative RD VKH - BABEL (1932) manifestations of same disease process - synonyms uveo-meningitic syndrome uveomeningoencephalic syndrome

VKH Chronic, Bilateral, Diffuse, Granulomatous Panuveitis with accompanying: Integumentary, Neurologic, Auditory involvement.

Incidence 1%-4% of all uveitis referrals in the US. 8% in Japan. Women appear to be affected more often than men except in the Japanese population.

Etiology Unknown, experimental evidence suggests a cell-mediated autoimmune process driven by T lymphocytes directed against self-antigens associated with melanocytes in genetically susceptible individuals.

However, the trigger (or triggers) of autoimmunity in genetically susceptible people remains unknown. Infectious agents, particularly viruses,may play a role in inducing the autoimmune response.

There are 4 - stages: - Prodromal, - Acute uveitic, - Convalescent, - Chronic recurrent.

Prodromal: Flulike symptoms. present with headache, nausea, meningismus,dysacusia, tinnitus, fever, orbital pain, photophobia. Skin and hair hypersensitivity to touch Focal neurologic disorder include cranial neuropathies, hemiparesis. Auditory problems are observed in 75% of patients.

Acute uveitic stage 1 to 2 days after the onset of central nervous systems Blurring of vision in both eyes. Bilateral granulomatous anterior uveitis. Vitritis, Choroidal thickening. Optic disc hyperemia. Multiple serous retinal detachments. Bullous exudative detachments. Profound visual loss Elevated IOP

Vogt-Koyanagi-Harada syndrome. A Vogt-Koyanagi-Harada syndrome. A. Fundus photograph of the left eye showing multifocal serous retinal detachment. B. Late fluorescein angiogram showing pooling of dye in the areas of serous detachment. C. Indocyanine angiography showing areas of blocked fluorescence corresponding to serous detachment. More areas of hypofluorescence are noted than those observed on fluorescein angiogram.

Convalescent stage Occurs several weeks later and is marked by resolution of the exudative retinal detachments gradual depigmentation of the choroid, resulting in the classic orange-red discoloration known as the sunset-glow fundus appearance

In addition, small, round, discrete depigmented lesions develop in the inferior peripheral fundus, representing resolved Dalen- Fuchs nodules Juxtapapillary depigmentation may also be seen

Perilimbal vitiligo (Sugiura sign) may be found in up to 85% of Japanese patients but is rarely observed among Caucasians

Integumentary changes Vitiligo, Alopecia, Poliosis, typically appear during the convalescent stage in about 30% of patients .

The convalescent phase usually lasts from 1 month to several years.

The recurrent or chronic recurrent phase Manifested by recurrent episodes of acute anterior granulomatous uveitis. Uveitis episodes usually respond poorly to corticosteroid based therapy, which affects only the anterior uvea.

Signs of chronic ocular inflammation, such as Busacca or Koeppe iris nodules, may be found. This inflammation is directly related to inadequate IMT.

complications Chronic usually seen in this phase include: Macular atrophy, Cataract, Glaucoma, CNV, Subretinal fibrosis, ERM, Optic atrophy

Photos of the right (2A) and left (2B) eyes show RPE alterations, loss of choroidal melanocytes and a resultant sunset-glow fundus, choroidal Dalen-Fuchs–like nodules, and subretinal fibrosis bilaterally.

Diagnostic Criteria complete, incomplete Probable

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