Purpose: To determine the most efficient diagnostic tools in Vogt-Koyanagi-Harada syndrome, taking into account the international diagnostic criteria, and to evaluate the therapeutic management of these patients.
Patients and methods: This study examined patients with a suspicion of VKH syndrome who presented between January 2001 and March 2003, including ocular and extraocular evaluation of the disease at the time of diagnosis. Each patient was classified according to the 1978 international diagnostic criteria and the revised 2001 criteria. In most cases, intravenous steroid pulses were administered. Immunosuppressors were initiated when inflammation was not controlled with steroids.
Results: Twenty-two patients were included. The mean age was 33.5 years (range, 15-49 years). Posterior segment involvement, which was observed in 21 patients, depended on the stage of the disease. Anterior segment inflammation was associated in eleven cases. Neurologic symptoms, including meningitis, cerebrospinal fluid lymphocytic pleocytosis, tinnitus, or hearing loss were observed in 12 patients. Fourteen patients had dermatologic signs. Five patients who developed VKH syndrome did not meet the 1978 criteria, and three patients did not meet the 2001 revised criteria. In 19 cases, intraocular inflammation was controlled with corticosteroids. In three cases, corticosteroids could not be discontinued. These patients were treated with immunosuppressive molecules: azathioprine, cyclophosphamide, interferon alpha. At the end of the follow-up period, inflammation was controlled in all patients.
Discussion: Revision of the diagnostic criteria provides a more subtle diagnosis of VKH syndrome. However, it is difficult to consider the variability of clinical symptoms during the duration of disease. Corticosteroids must be used at appropriate dosages, followed by slow tapering over 6 months. This attitude seems to reduce the duration of ocular inflammation and decreases the frequency of recurrence. The use of immunomodulating drugs could be reduced by early and appropriate use of systemic steroids. Interferon alpha seems to be a promising alternative in corticoresistant or corticodependent forms of the disease, but further controlled studies are required.