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VogteKoyanagieHarada (VKH) disease is a bilateral, chronic granulomatous panuveitis associated with central nervous system, auditory, and integumentary manifestations. Classically, the disease begins with a prodromal phase of neuroauditory symptoms, followed by an acute uveitis phase, and finally, the chronic stage manifestations. However, it is not uncommon for patients with initial-onset VKH disease to present with the isolated ocular disease, without associated neuroauditory symptoms. Any delay in establishing the correct diagnosis of initial-onset VKH disease and in initiating adequate treatment may result in higher risks of chronicity, complications, and visual impairment. Therefore, accurate recognition of the distinctive ocular features associated with the initial-onset VKH will help to establish an early definitive diagnosis, with prompt initiation of appropriate treatment. Several criteria have been proposed to clarify the diagnostic approach for VKH disease. In 1978, Sugiura suggested a set of criteria for the diagnosis of VKH disease. Bilateral ocular inflammation, especially with posterior manifestations, along with typical fluorescein angiography findings and pleocytosis of the cerebrospinal fluid were required criteria for the diagnosis. Although this was an immense step in outlining the features of VKH, his criteria were not sensitive enough to diagnose patients after the acute presentation. Moreover, the necessity of obtaining cerebrospinal fluid limited its application in many countries. Two years later, the American Society of Uveitis (AUS) redefined Sugiura’s criteria by detailing the posterior uveitis manifestations and extending the items of

“Revised diagnostic criteria” for Vogt-Koyanagi-Harada disease fail to improve disease management