Vogt–Koyanagi–Harada syndrome

Vogtâ€"Koyanagiâ€"Harada syndrome (VKH syndrome) is an uncommon multisystem disease of presumed autoimmune etiology that is characterized by chronic, bilateral, diffuse, granulomatous uveitis with accompanying dermatologic, neurologic, and auditory involvement. VKH syndrome is named for ophthalmologists Alfred Vogt from Switzerland and Yoshizo Koyanagi and Einosuke Harada from Japan.

Pathophysiology

VKH syndrome is an immune-mediated disease. The mechanism of the disease is thought to be T helper cell mediated autoimmune attack of melanocytes in the skin, uvea, central nervous system and inner ear. A new T-cell subset Th17 may play an important role in the initiation and maintenance of inflammatory disease when stimulated by the interleukin IL-23, thus producing IL-17.

There is a higher rate of VKH syndrome in people of Asian, Latin, and Mediterranean descent

Clinical presentation

There are 4 stages of VKH syndrome: prodromal, acute uveitic, convalescent, and chronic recurrent.

• The prodromal stage is marked by flu-like symptoms. Patients present with headache, nausea, meningism, dysacusia (discomfort caused by loud noises or a distortion in the quality of the sounds being heard) and tinnitus.
• The acute uveitic stage is heralded by the onset of blurring of vision in both eyes and is marked by bilateral granulomatous anterior uveitis, variable degree of vitritis, thickening of the posterior choroid with elevation of the peripapillary retinal choroidal layer, hyperemia and edema of the optic nerve, and multiple serous retinal detachments.
• The convalescent stage occurs several weeks later and is marked by gradual depigmentation of the choroid, resulting in the classic orange-red discoloration, or sunset-glow fundus appearance. Skin changes including alopecia, vitiligo, and poliosis typically appear during this stage.
• The chronic recurrent stage is marked by repeated bouts of uveitis.

Diagnosis

The diagnosis of VKH syndrome is made by ophthalmologist or optometrist based on clinical presentation. Imaging techniques such as fluorescein angiography are used to confirm diagnosis.

Treatment

The acute stage of VKH syndrome is exquisitely responsive to early and aggressive treatment with topical, periocular and systemic corticosteroids and cycloplegic agents.

References

External links

• VKH Story

Share on Google Plus

About Unknown

This is a short description in the author block about the author. You edit it by entering text in the "Biographical Info" field in the user admin panel.