Vogt-Koyanagi-Harada (VKH)-What Do We Know About the Disease, and Can We Recognize It?

Background

Vogt-Koyanagi-Harada (VKH) is a multisystem autoimmune disease that ophthalmologists often encounter first. The condition is caused by an immune response against tyrosinase-related proteins in pigment cells (melanocytes) of the uvea, inner ear, meninges, and skin, and the process may be triggered by genetic and environmental factors. Although much is known about the disease, establishing an accurate and timely diagnosis still requires a multidisciplinary team and strong clinical expertise. Treatment demands early and aggressive anti-inflammatory therapy with corticosteroids, often prolonged and combined with immunosuppressive or biological agents. Aim: The present article aims to present three unique cases of patients with VKH syndrome, diagnosed and monitored by Ophthalmologists using standard imaging techniques over the course of five years, to demonstrate the unusual manifestations of the already rare syndrome and to improve the general knowledge of the disease among Ophthalmology specialists.

Methods

Three different patients with various subjective symptoms and unique clinical signs went through observation in University Specialized Eye Hospital for Active Treatment-Varna.

Results

The three clinical cases presented diagnostic challenges, the key role of imaging studies and the importance of thorough medical history taking.

Conclusions

The prognosis in VKH is variable-timely diagnosis and treatment are essential to reduce the risk of recurrence and chronic progression of the disease.