Long-term Outcomes of Limited Vogt-Koyanagi-Harada Syndrome

To describe the long-term outcomes of patients with limited Vogt-Koyanagi Harada syndrome (VKH), characterized by steroid-responsive serous retinal detachments without other signs of intraocular inflammation.

Retrospective case series.

Consecutive patients from the Royal Victorian Eye and Ear Hospital with acute bilateral serous retinal detachments without anterior chamber inflammation, with no previous ocular history or trauma, and with negative screening for vasculitis and other systemic autoimmune disease were included. Serous retinal detachment was confirmed on optical coherence tomography (OCT) and fundus fluorescein angiography. Visual acuity, treatment regimen, complications, and duration of follow-up were recorded.

Nine patients (6 male, median age 29 years [interquartile range (IQR) 27-36 years]) from January 2010 through May 2014 were studied. Median presenting logMAR visual acuity (VA) was 0.48 (IQR -0.1 to 3.0). Six patients were initially commenced on intravenous methylprednisolone for 3 days, followed by oral prednisolone. All received a course of tapering oral prednisolone (1 mg/kg prior to taper). Median duration of treatment was 9 weeks (IQR 8-14 weeks). Median time to complete resolution of subretinal fluid on OCT was 3.7 weeks (range, 2-12 weeks), with a corresponding improvement in VA (median logMAR 0.00; range, -0.08 to 0.04). There was no recurrence after a mean follow-up of 145.2 weeks (95% confidence interval 72.5-217.9).

In contrast to typical VKH, patients with limited VKH in our series appear to have good outcomes with systemic corticosteroid treatment. They have a marked improvement of VA and no episodes of relapse after cessation of corticosteroid treatment.