raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:279928

Paraneoplastic uveitis

Type
Disease
Group
Disorder
Age of onset
Adult
Status
active

A rare ophthalmic disorder characterized by a non-infectious uveitis potentially involving all parts of the uveal tract and due to a paraneoplastic autoimmune reaction. The signs and symptoms usually precede the tumor diagnosis and may include ocular redness and pain, light sensitivity, blurred vision, floaters, and decreased vision. The most classic form is the intermediate uveitis associated with anti-CV2 antibodies in small-cell lung cancer or other neoplasms. Other ocular and/or extra-ocular manifestations are commonly present in addition.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enParaneoplastic uveitis
frUvéite paranéoplasique
deUveitis, paraneoplastische
esUveítis paraneoplásica
itUveite paraneoplastica
nlParaneoplastische uveïtis
ptUveíte paraneoplásica
plParaneoplastyczne zapalenie błony naczyniowej oka
csParaneoplastická uveitida
ukПаранеопластичний увеїт
zh副肿瘤性葡萄膜炎
trParaneoplastik üveit

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10H57.8NTBTattributedvalidated
ICD-119B65.0NTBTattributedvalidated
MONDO0017212Evalidated
UMLSC5680786Evalidated

E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.

3

Classification

Preferential parent (Orphanet linearisation):

Parents in the classifications (1):

No children.

4

Genes

0 direct

Orphanet records no gene directly on this code.

5

Prevalence and incidence

2 records
TypeClassValueAreaQualificationValidationSource
Cases/families10 casesWorldwideCase(s)Validated41003888[PMID]
Point prevalence<1 / 1 000 000—WorldwideClass onlyValidated41003888[PMID]_ORPHANET

Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.

6

HPO phenotypes

Orphanet has no HPO annotation for this code in this release.

7

Sources for this card