raremaprare-disease reference indexOrphadata 2026-06-23 · HPO 2026-09-01
ORPHA:499103

Recurrent idiopathic neuroretinitis

Synonyms: RINR

Type
Disease
Group
Disorder
Inheritance
Not applicable
Status
active

A rare inflammatory optic neuropathy characterized by recurrent episodes of idiopathic inflammation of the optic nerve head with optic disc edema associated with macular exudate in a star-shaped pattern. Patients present with acute visual loss, most typically in the form of a large central scotoma. Pain is mild or absent. Bilateral involvement is frequent and usually sequential. The interval between attacks is highly variable, ranging from months to several years. Visual loss is cumulative with each attack and often severe.

1

Names in Orphadata languages

12 of 12 languages
LangNameSynonyms
enRecurrent idiopathic neuroretinitisRINR
frNeurorétinite idiopathique récurrente
deNeuroretinitis, idiopathische, rezidivierende= en
esNeurorretinitis idiopática recurrente= en
itNeuroretinite idiopatica ricorrente
nlTerugkerende idiopathische neuroretinitis= en
ptNeurorretinite idiopática recorrente
plIdiopatyczne powtarzające się zapalenie nerwu wzrokowego= en
csRekurentní idiopatická neuroretinitida= en
ukРецидивуючий ідіопатичний нейроретиніт= en
zh反复发作的特发性视神经视网膜炎= en
trTekrarlayan idiyopatik nöroretinit= en

Definition in:

2

Cross-references

qualifier = Orphanet mapping relation
SourceCodeQualifierICD relationValidation
ICD-10H46NTBTattributedvalidated
ICD-119C40.2NTBTattributedvalidated
MONDO0044689Evalidated
UMLSC5680094Evalidated

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

0 records

No prevalence record in this release.

6

HPO phenotypes

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

7

Sources for this card