Rare autoimmune syndrome behind a young man's sudden vision loss

A 25-year-old Black man arrived at the Tufts Medical Center ED with progressive blurred vision in both eyes — starting in the left, then spreading to the right four days later — following a recent bout of fever and headache initially dismissed as a migraine. After an extensive workup ruling out infections (syphilis, TB), autoimmune conditions, and neoplastic causes, imaging told the real story: bilateral serous retinal detachments with bacillary layer detachment on OCT, pinpoint hyperfluorescence on fluorescein angiography, and choroidal thickening on ultrasound — a classic picture of Vogt-Koyanagi-Harada (VKH) syndrome.

VKH is a rare autoimmune disorder in which T cells attack melanocyte antigens in the uveal tract, meninges, inner ear, and skin. It progresses through four phases — prodromal (flu-like illness), acute uveitic, convalescent (pigment loss), and chronic recurrent — and disproportionately affects Hispanic, Asian, Native American, and Middle Eastern populations. The patient was started on oral prednisone 80 mg/day, with near-complete resolution of retinal fluid in just three days and full 20/20 vision restored at four months.

Key Takeaways:

• First-line treatment is high-dose systemic corticosteroids; intravitreal injections and PDT are not appropriate for acute VKH
• Immunomodulatory therapy (e.g., methotrexate, mycophenolate mofetil) should be considered early for persistent vision loss or recurrence
• Mycophenolate mofetil has shown 93% of patients achieving 20/20 vision, with 58% relapse-free after discontinuation

Why it matters: VKH is easily misdiagnosed — this patient was initially sent home with a migraine diagnosis. Recognizing its hallmark imaging findings and initiating systemic steroids promptly can mean the difference between full visual recovery and permanent damage.