PURPOSE: To describe an atypical late-onset presentation of Vogt-Koyanagi-Harada (VKH) disease initially interpreted as autoimmune glial fibrillary acidic protein astrocytopathy (GFAP-A) in association with an ovarian teratoma. METHODS: A 59-year-old woman presented with bilateral optic disc edema, visual field defects, and headache following a flu-like illness. Comprehensive ophthalmologic, neurologic, and systemic evaluation was performed, including multimodal ocular imaging, cerebrospinal fluid (CSF) analysis with antibody testing, and neuroimaging. Clinical course and response to corticosteroid therapy were assessed longitudinally. RESULTS: Initial findings, including CSF anti-GFAP positivity, lymphocytic pleocytosis, white matter lesions, and an ovarian teratoma, supported a diagnosis of GFAP-A. However, sequential multimodal imaging revealed diffuse, steroid-responsive choroidal inflammation with choroidal thickening, folds, mild subretinal fluid, hypocyanescent dark dots on indocyanine green angiography, fluorescein angiography pinpoints, and bilateral hot discs. Granulomatous anterior uveitis and subsequent choroidal depigmentation further supported VKH. Bilateral sensorineural hearing loss was also consistent with this diagnosis. The longitudinal ocular phenotype proved more consistent with VKH than with primary astrocytopathy-related optic neuropathy. CONCLUSIONS: Late-onset VKH may present predominantly with optic disc edema and central nervous system involvement, closely mimicking inflammatory neurologic disease. Recognition of subtle choroidal inflammation and integration of longitudinal multimodal imaging are essential to avoid misdiagnosis and guide appropriate immunosuppressive management.
Goncharova-Simón et al. (Tue,) studied this question.
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