[VOGT-KOYANAGI-HARADA SYNDROME].

Abstract

Introduction: Vogt-Koyanagi-Harada (VKH) syndrome is an autoimmune disease characterized at its acute stage by pan-ocular granulomatous inflammation. Systemic symptoms may develop, including hearing impairment, neurologic disorders, and loss of pigment in the skin and hair. Ocular complications occur during the chronic stage or secondary to flare-ups and may result in substantial vision loss due to the intraocular inflammation. This manuscript presents a case report and literature review of the ocular and systemic manifestations, diagnostic criteria, differential diagnosis and treatment considerations in VKH. We describe a case of a 14-year-old patient who presented with bilateral granulomatous uveitis with optic nerve and central retinal edema, with no other complaints. She underwent workup to rule out a systemic disease including an infectious and rheumatologic disease, which was unremarkable. She was treated with systemic steroids but suffered from worsening of her symptoms. She subsequently developed imaging manifestations consistent with VKH. Therefore, she was also treated with immunosuppressants including Imuran and Cyclosporin, as well as biological therapy due to recurring flare-ups, with good response. Early diagnosis and appropriate treatment of VKH is important to minimize ocular and extraocular complications.