Vogt–Koyanagi–Harada syndrome, also called uveomeningitis syndrome and uveomeningoencephalitic syndrome,it is a multisystem disease of presumed autoimmune cause, that affects pigmented tissues, which have melanin. The most significant manifestation is bilateral, diffuse uveitis, which affects the eye. VKH may variably also involve the inner ear with effects on hearing, the skin, and the meninges of the central nervous system
Vogt-Koyanagi-Harada disease (VKHD) is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin and hair. This disease is mainly a lymphocyte mediated aggression to melanocytes after a viral trigger in the presence of HLA-DRB1. The absence of ocular trauma or previous intraocular surgery sets VKHD. The disease has an acute onset of bilateral blurred vision with hyperemia preceded by flu-like symptoms. The acute uveitic stage is characterized by a diffuse choroiditis with serous retinal detachment and optic disc hyperemia and edema. Fluorescein angiography in this phase demonstrates multiple early hyperfluorescent points. After the acute uveitic stage, ocular and integumentary system pigmentary changes may appear. Ocular findings may be accompanied by lymphocytic meningitis, hearing impairment and/or tinnitus in a variable proportion of patients
Patients with Vogt-Koyanagi-Harada disease usually initially present to an ophthalmologist for ocular problems, including sudden loss of vision, ocular pain and photophobia. Hearing disturbances and dizziness may be present. After weeks or months, most patients notice cutaneous signs (e.g. hair loss, poliosis, vitiligo).
B-mode ocular ultrasound features of Vogt-Koyanagi-H
MR imaging may detect early CNS involvement by Vogt-Koyanagi-Harada disease before the onset of neurologic symptoms which include:
• typical bilaterality of ocular findings
• scattered periventricular white matter lesions on T2-weighted imaging/FLAIR, and pachymeningeal enhancement.
• bilateral contrast enhancement of the choroid is seen along diffuse choroidal thickening with scleral sparing and retinal detachment
• . Prompt diagnosis followed by early, aggressive and long-term treatment with high-dose corticosteroids is most often ensued by good visual outcomes. However, some patients may experience chronic uveal inflammation with functional eye deterioration.့့