Clinical features and visual outcomes of 111 patients with new-onset acute Vogt-Koyanagi-Harada disease treated with pulse intravenous corticosteroids

Purpose To describe the clinical features, treatment and visual outcomes of Japanese patients with new-onset acute Vogt-Koyanagi-Harada (VKH) disease. Methods Clinical records of 111 patients who presented between 1999 and 2015 to the Ocular Inflammation Service of the Kyorin Eye Center, Tokyo, were reviewed. Results Of the 111 patients (68 women, 43 men), 16 had complete, 90 had incomplete and 5 had probable VKH disease. The median follow-up period was 36 months (4-175 months). The mean age at presentation was 41 years (19-74 years). Serous retinal detachment (202 eyes) and optic disc hyperaemia (89 eyes) were observed at presentation. Of the patients tested, 45/48 (93.8%) were human leucocyte antigen-DR4 positive and 63/77 (81.8%) had cerebrospinal fluid pleocytosis. Initial corticosteroid treatment consisted of pulse intravenous therapy in all patients. Sunset glow fundus was observed in 49.5% of eyes, and anterior and/or posterior segment recurrence of inflammation was observed in 25 patients (22.5%). Treatment was transitioned to cyclosporine in 17 patients (15.3%) for steroid sparing (6 patients) or recurrent inflammation (11 patients), with good subsequent control. Ocular complications were observed in 47 of 222 eyes (21.2%) (mostly cataract), and systemic complications were observed in 8.1% of patients (mostly hypertension and diabetes mellitus). Ninety-three percent of eyes (167 of 178 eyes) had a visual acuity of >= 1.0 at 1 year after presentation. Conclusions An aggressive corticosteroid treatment strategy in a large number of patients with new-onset acute VKH disease, with transitioning to cyclosporine in selected cases, resulted in excellent visual outcomes and low rates of recurrence.