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

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.1 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.2,3 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. 4 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 neuroauditory signs.5 The AUS criteria did not include pleocytosis as a required criterion for diagnosis but considered it part of the neuroauditory signs. Because sympathetic ophthalmia was not clinically distinguishable from VKH, “lack of history of trauma or surgery” was set as a prerequisite for diagnosis. However, the fluorescein angiography findings, which were an important clue during the acute stage of disease, failed to be mentioned. Similar to Sugiura’s criteria, the AUS criteria failed to differentiate between acute and chronic stages of the disease. In 1999, the first VKH International Workshop group proposed the revised diagnostic criteria for VKH disease. It aimed to overcome the shortcomings of previous criteria, in particular, boosting up the sensitivity and specificity.6 The ocular manifestations were described in more detail and fluorescein angiography, and ultrasonography findings were adopted as useful ancillary tests for the diagnosis. Moreover, it categorized the VKH patients into three groups: (1) complete, (2) incomplete, and (3) probable based on the presence of ocular and extraocular manifestations. Our purpose here is to explain, based on recent data, why the revised diagnostic criteria for VKH disease failed to improve management and should be reconsidered from the perspective of reaching a rapid diagnosis of initial-onset disease. Limitations of the revised criteria currently include: (1) not clearly differentiating initial-onset disease from chronic disease, which has a crucial impact on management; (2) division into three groups, which have very little clinical relevance; and (3) not considering highly sensitive investigations such as indocyanine green angiography (ICGA), spectral domain optical coherence tomography (SD-OCT), and high-penetration imaging techniques of OCT [enhanced depth imaging optical coherence tomography (EDI-OCT) and swept source OCT