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

Abstract

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.

Keywords: Vogt-Koyanagi-Harada disease; corticosteroids; cyclosporine; treatment; visual outcome.