Incidence and clinical features of recurrent Vogt-Koyanagi-Harada disease in Japanese individuals

Purpose Our aim was to determine the recurrence rate of inflammation in Vogt-Koyanagi-Harada (VKH) disease and to describe its clinical features. Methods We retrospectively evaluated patients diagnosed as having VKH disease with exudative retinal detachment at the Osaka University Hospital or the Japanese Community Healthcare Organization, Osaka Hospital, between 1998 and 2012. All patients received high-dose corticosteroid therapy as the initial treatment and were followed for at least 6 months. Demographic data, including age, sex, visual acuity (VA) levels at initial presentation and at 1 year after initial therapy, treatment received, and recurrent episodes were reviewed. Results Fifty-five consecutive patients with VKH disease were identified (36 women; mean age 38.6 ± 10.4 years). Fourteen patients (25.5 %) had recurrent inflammation, which manifested as posterior uveitis in eight and as anterior uveitis in six of the patients. Recurrent posterior segment inflammation was more likely to develop in patients whose VA at initial presentation was poor ( P = 0.039) and in whom orally administered corticosteroid was tapered rapidly (to 30 mg within 3 weeks or less, to 20 mg within 2 months or less, and to 10 mg within 3 months or less) ( P = 0.006, P = 0.066, and P = 0.041, respectively). Conclusions About 25 % of patients with VKH disease had recurrent inflammation. Poor initial VA and rapid tapering of the corticosteroid were associated with posterior recurrence.