Idiopathic panuveitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Status
- active
Idiopathic panuveitis is a rare inflammatory eye disease, of unknown etiology, characterized by generalized inflammation of the uvea (iris, ciliary body, choroid), retina and vitreous with consequent ciliary spasm and posterior synechiae formation, leading to acute or chronic, unilateral or bilateral visual impairment and ocular discomfort or pain. Patients present an increased risk of development of cataracts, secondary glaucoma, cystoid macular edema and/or retinal detachment. It could potentially result in vision loss.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Idiopathic panuveitis | |
| fr | Panuvéite idiopathique | |
| de | Panuveitis, idiopathische | |
| es | Panuveítis idiopática | |
| it | Panuveite idiopatica | |
| nl | Idiopathische panuveïtis | |
| pt | Panuveíte idiopática | |
| pl | Idiopatyczne zapalenie wszystkich struktur błony naczyniowej | |
| cs | Idiopatická panuveitida | |
| uk | Ідіопатичний панувеїт | |
| zh | 特发性全葡萄膜炎 | |
| tr | İdiyopatik panüveit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | H44.1 | NTBT | attributed | validated |
| ICD-11 | 9C20.0 | NTBT | attributed | validated |
| MONDO | 0017258 | E | validated | |
| UMLS | C4707884 | E | validated |
E exact · NTBT this ORPHAcode is narrower than the target · BTNT broader than the target · ND not decided.
Classification
Preferential parent (Orphanet linearisation):
Parents in the classifications (1):
- ORPHA:280898Panuveitis
rare ophthalmic disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
4 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | 1-9 / 100 000 | 2.0194 / 100 000 | Europe | Value and class | Validated | ORPHANET |
| Annual incidence | 1-9 / 1 000 000 | 0.5051 / 100 000 | Europe | Value and class | Validated | ORPHANET |
| Point prevalence | 1-5 / 10 000 | 16 / 100 000 | United States | Value and class | Validated | 27608193[PMID] |
| Annual incidence | 1-9 / 100 000 | 3.6 / 100 000 | United States | Value and class | Validated | 34802369[PMID] |
Value is Orphadata's ValMoy: the mean estimate per 100 000 for prevalence and incidence, the number of cases or families for case reports.
HPO phenotypes
19 annotations · validated 2019-07-09| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Frequent (79-30%) | ||
| HP:0000504 | Abnormality of vision | |
| HP:0000622 | Blurred vision | |
| HP:0200026 | Ocular pain | |
| HP:0025337 | Red eye | |
| HP:0007663 | Reduced visual acuity | |
| HP:0030652 | Vitreous haze | |
| Occasional (29-5%) | ||
| HP:0000518 | Cataract | |
| HP:0030953 | Conjunctival hyperemia | |
| HP:0011505 | Cystoid macular edema | |
| HP:0002315 | Headache | |
| HP:0000616 | Miosis | |
| HP:0000613 | Photophobia | |
| HP:0011484 | Posterior synechiae of the anterior chamber | |
| HP:0100832 | Vitreous floaters | |
| HP:0030661 | Vitreous snowballs | |
| Very rare (<4-1%) | ||
| HP:0000618 | Blindness | |
| HP:0011506 | Choroidal neovascularization | |
| HP:0100014 | Epiretinal membrane | |
| HP:0007906 | Ocular hypertension | |
Annotation source: 19902397[PMID]_27782762[PMID]_2795939[PMID]_31082037[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/280921
- This record as JSON: /data/d/280921.json