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

**Authors:** Maria Boyadzhieva, Preslava Encheva, Dobrin Boyadzhiev, Valeri Sheherov, Darina Koseva, Zornitsa Zlatarova

PMC · DOI: 10.3390/diagnostics16010141 · Diagnostics · 2026-01-01

## TL;DR

This paper discusses Vogt–Koyanagi–Harada (VKH), a rare autoimmune disease, through three unique patient cases to improve ophthalmologists' understanding and diagnosis of the condition.

## Contribution

The paper contributes three unique clinical cases of VKH syndrome to highlight unusual manifestations and emphasize the importance of imaging and detailed history in diagnosis.

## Key findings

- VKH diagnosis requires a multidisciplinary approach and relies heavily on imaging and clinical history.
- Early and aggressive anti-inflammatory treatment is crucial to prevent chronic progression of VKH.
- Unusual clinical presentations of VKH can challenge accurate and timely diagnosis.

## Abstract

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.

## Linked entities

- **Diseases:** VKH syndrome (MONDO:0018092)

## Full-text entities

- **Genes:** TYR (tyrosinase) [NCBI Gene 7299] {aka ATN, CMM8, OCA1, OCA1A, OCAIA, SHEP3}
- **Diseases:** inflammatory (MESH:D007249), autoimmune disease (MESH:D001327), VKH (MESH:D014607)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Full text

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## Figures

18 figures with captions in the complete paper: https://tomesphere.com/paper/PMC12785815/full.md

## References

22 references — full list in the complete paper: https://tomesphere.com/paper/PMC12785815/full.md

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Source: https://tomesphere.com/paper/PMC12785815