How to identify, manage Vogt-Koyanagi-Harada disease

Editor's Note: Vogt-Koyanagi-Harada (VKH) disease is an uncommon but serious inflammatory disorder directed against melanin-containing cells in the uveal tract, meninges, inner ear, and skin. These observations, together with recent studies that have identified specific auto-antigens in melanin-containing cells, support the idea that VKH disease is an autoimmune disorder.

Treatment choices are often complicated by the chronic, recurrent nature of the inflammation. Early therapy almost always includes a combination of topical and systemic corticosteroids together with a topical cycloplegic mydriatic agent. Longer-term control frequently requires the use of one or more noncorticosteroid immunosuppressive agents to minimize the risk of both corticosteroid-induced side effects and recurrences.

A panel of uveitis specialists, including myself, commented on aspects related to the diagnosis and management of VKH disease in this first of a two-part series.

Who gets VKH disease, and what are the historical and clinical clues that help make the diagnosis? Can VKH disease occur without serous retinal detachments? Does it always have to be granulomatous?

Emmett T. Cunningham Jr., MD, PhD, MPH: Uveitis is present in all patients with VKH disease and is typically chronic, bilateral, and diffuse. Multiple, bilateral serous retinal detachments are the hallmark of the disorder but may be absent in a minority of patients, particularly early in the course of the disease and during recurrences.

Early neurologic symptoms of headache, meningismus, dysacousis, and/or tinnitus are often present. Late dermatologic signs include vitiligo, poliosis, and alopecia, but are less common and may be avoided entirely when patients are given prompt and aggressive therapy. Virtually all patients are of Asian, Asian Indian, or American Indian origin, and there is a strong association between VKH disease and the class II HLA-DR4 haplotype.

Ralph D. Levinson, MD: VKH disease can be found in children as well as adults. Populations at risk appear to be primarily Asians and groups that may have clustered along the Silk Route (individuals from the Middle East or North Africa) and over the land bridge to North America (Native American and Mestizo individuals). I have most often seen it in individuals of Mestizo descent in their third decade, but that is due to the population at risk in my practice.

However, physicians at the National Eye Institute have reported that a large proportion of their patients with VKH disease were African-American (which may in part be due to the fact that African-Americans may have some Native American ancestry), and it has been suggested that the population at risk may reflect, at least in part, other (unknown) factors more common in heavily pigmented populations.

While classified most often as a granulomatous panuveitis, the uveitis may not appear granulomatous if treated early and aggressively.

The diagnostic criteria have recently been revised (Table 1). It is primarily a clinical diagnosis. The only laboratory testing specifically mentioned in these criteria is cerebrospinal fluid (CSF) pleocytosis, which is not required for the diagnosis, and it is stated that there should be no laboratory evidence of other disease, but no specific battery of tests is required.