Features
Vogt-Koyanagi-Harada (VKH) syndrome is a multisystem inflammatory condition affecting the eye, skin, inner ear, and meninges. The condition is characterized by ocular inflammation in association with patchy depigmentation of the skin (▶ Fig. 72.1), patchy hair loss, and whitening of hair (in particular the eyelashes). VKH syndrome more commonly affects those with pigmented skin and of certain genetic predisposition. The prevalence varies and is more common in Asia, Latin America, and the Middle East. In the United States, VKH accounts for approximately 3 to 7% of uveitis referrals and Hispanics are most frequently affected. The mean age at presentation is 32 to 35 years of age, though it has been reported in children as young as 3 years.
Fig. 72.1 (a,b) Vitiligo with a symmetric distribution.
Immunological and histopathological studies suggest that CD4+ T cells targeting melanocytes may be the primary factor initiating the inflammatory process seen in VKH. Genetic susceptibility in patients expressing HLA DRB1*0405 may also predispose some patients, possibly in combination with a viral trigger.
VKH is a systemic disorder and the extraocular manifestations are critical in the diagnosis. The revised criteria for the diagnosis of VKH categorized the disease into definite (complete and incomplete) and probable diagnosis. The syndrome evolves through four distinct phases, including prodromal, uveitic, convalescent, and chronic recurrent phase.
72.1.1 Common Symptoms
Prodromal
Nonspecific symptoms, typically lasting 3 to 5 days; most commonly headaches and meningismus. Photophobia, orbital pain, vertigo, nausea, weakness, and flulike symptoms (including fever) are frequently reported. Hearing disturbances such as tinnitus and sensory hearing loss are common (typically involving the higher frequencies). Dysacousia may persist for years; focal neurologic deficits, including cranial nerve palsies and optic neuritis, have been reported; pleocytosis of cerebrospinal fluid can be seen in more than 80% of patients and can last up to 8 weeks; some patients report sensitivity of hair and skin to touch.
Uveitic
This phase lasts for several weeks; although some patients may experience a delay before the onset of symptoms in the second eye, most patients present with bilateral vision loss as the most common presenting symptom.
72.1.2 Exam Findings
Uveitic
Ocular presentation is an acute, bilateral granulomatous panuveitis in up to 70% of patients characterized by diffuse, increased choroidal thickening, multifocal detachments of the sensory retinal, and optic nerve edema (▶ Fig. 72.2, ▶ Fig. 72.3, ▶ Fig. 72.4, ▶ Fig. 72.5). Inferior pooling of subretinal fluid can be seen. Early in the disease, shallowing of the anterior chamber with an increased intraocular pressure may result from edema of the ciliary processes. Anterior segment findings may include mutton-fat keratic precipitates, iris nodules, pupillary membranes, or diffuse iris thickening.
Fig. 72.2 (a,b) Optical coherence tomography in acute Vogt-Koyanagi-Harada uveitis demonstrating multifocal subretinal fluid.
Fig. 72.3 Acute Vogt-Koyanagi-Harada (VKH) angiographic and fundus imaging. (a) Fundus photography. (b–d) Characteristic indocyanine green angiography findings during acute VKH. (e,f) Fluorescein angiography showing pinpoint areas of hyperfluorescence with late pooling.
Fig. 72.4 (a,b) Enhanced-depth imaging optical coherence tomography (OCT) in Vogt-Koyanagi-Harada. Imaging demonstrates increased choroidal thickening without fluid on enhanced-depth OCT in chronic, recurrent subclinical disease.
Fig. 72.5 Multimodal imaging in active Vogt-Koyanagi-Harada (VKH). (a) Chorioretinal folds, serous detachment, and inferior pooling of subretinal fluid with serous detachment on ultra-widefield imaging. (b) Chorioretinal folds are more visible on macular view. (c,d) Wide-field fluorescein angiography shows numerous pinpoint areas of leakage with multifocal late pooling of subretinal dye in acute VKH—“starry sky” appearance. (e,f) Subretinal fluid and chorioretinal folds on optical coherence tomography.
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