Vogt-Koyanagi-Harada disease presenting as acute angle closure glaucoma at onset

Background: To investigate the clinical features of Vogt-Koyanagi-Harada (VKH) disease presenting as acute angle closure glaucoma at onset. Design: Retrospective non-comparative case series. Participants: Four hundred and eighty-six VKH patients seen from February 2001 to March 2010. Methods: The history and clinical findings of all patients were reviewed. Auxiliary examinations, including ultrasound biomicroscopy, fundus fluorescein angiography and optical coherence tomography, were performed in certain cases. Corticosteroids with or without cyclosporine A were used to treat these patients. Main Outcome Measure: Patients' demographics, clinical presentation and auxiliary examination findings. Results: Eight out of 486 VKH patients were misdiagnosed as acute angle closure glaucoma. The mean age of these eight patients was 55.6 years. Six patients were female. The mean intraocular pressure (IOP) at disease onset was 32.9 mmHg. All of these patients had a shallow anterior chamber and a narrow or closed angle at their first visit. The complaints of these patients were mostly headache and sudden decreased vision in both eyes. Alterations shown on ultrasound biomicroscopy included detachment of the ciliary body and peripheral choroid. The increased IOP did not respond to anti-glaucoma therapy, but resolved following treatment with corticosteroids. The eye of one patient was enucleated after failed trabeculectomies prior to referral to our uveitis centre. Conclusion: VKH disease presenting with a bilateral increased IOP mostly occurs in older women. The strikingly decreased visual acuity associated with mild to moderate increased IOP is a clue to the diagnosis. The increased IOP responded well to corticosteroids but not to anti-glaucoma treatment.