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Start free with EleplanVogt-Koyanagi-Harada disease
ORPHA:3437Disease
Also called Uveomenigitic syndrome
What it is
A rare bilateral granulomatous panuveitis characterized by an acute phase with serous retinal detachments associated with neurological (meningitis) and auditory alterations which, in case of delayed (>3-4 weeks) or insufficient treatment, evolves into a chronic phase with chronic anterior uveitis and retinal atrophic lesions (Dalen-Fuchs nodules and sunset glow fundus) associated with dermatological alterations. The disease is marked by increased choroidal inflammatory activity.
Key facts
- Prevalence
- 1-9 / 1 000 000 (annual incidence, United States)
- Age of onset
- All ages
- Inheritance
- Multigenic/multifactorial
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
10Common30–79%
15Sometimes5–29%
12and 4 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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