Fuchs heterochromic iridocyclitis
Synonyms: FHI
- Type
- Disease
- Group
- Disorder
- Age of onset
- Adult
- Status
- active
Fuchs heterochromic iridocyclitis (FHI) is an ocular disease of unknown etiology occurring in a very small percentage (0.5-6.2%) of uveitis cases, characterized by diffuse iris heterochromia or atrophy, keratic precipitates in the absence of synechiae, and in some cases evolving to glaucoma and vitreous opacities.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Fuchs heterochromic iridocyclitis | FHI |
| fr | Iridocyclite hétérochromique de Fuchs | = en |
| de | Fuchs Heterochromie-Iridozyklitis | Fuchs'sche Uveitis-Syndrom · Heterochromiezyklitis Fuchs |
| es | Iridociclitis heterocrómica de Fuchs | |
| it | Iridociclite eterocromica di Fuchs | = en |
| nl | Heterochrome iridocyclitis van Fuchs | = en |
| pt | Iridociclite heterocrómica de Fuchs | |
| pl | Heterochromiczne zapalenie tęczówki i ciała rzęskowego Fuchsa | = en |
| cs | Fuchsova heterochromní iridocyklitida | = en |
| uk | Гетерохромний іридоцикліт Фукса | = en |
| zh | Fuchs异色性虹膜睫状体炎 | = en |
| tr | Fuchs heterokromik iridosiklit | = en |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | H20.8 | NTBT | index | validated |
| ICD-11 | 9A96.1 | NTBT | index | validated |
| MONDO | 0016989 | E | validated | |
| UMLS | C0016782 | E | validated | |
| GARD | 6791 | E | validated | |
| MedDRA | 10017406 | 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:306648Non-infectious anterior uveitis
rare ophthalmic disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
3 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Annual incidence | 1-9 / 1 000 000 | 0.2 / 100 000 | Finland | Value and class | Validated | 9088407[PMID] |
| Point prevalence | 1-9 / 1 000 000 | 0.5 / 100 000 | Finland | Value and class | Validated | 9088407[PMID] |
| Point prevalence | 1-9 / 1 000 000 | — | Europe | Class only | Not yet validated | 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.
HPO phenotypes
24 annotations · validated 2023-03-31| HPO | Term (as annotated by Orphanet) | Diagnostic |
|---|---|---|
| Very frequent (99-80%) | ||
| HP:0031701 | Anterior chamber inflammatory cells | |
| HP:0025341 | Corneal keratic precipitates | |
| HP:0001089 | Iris atrophy | |
| HP:0100832 | Vitreous floaters | |
| Frequent (79-30%) | ||
| HP:0000622 | Blurred vision | |
| HP:0034821 | Bussaca nodules | |
| HP:0000518 | Cataract | |
| HP:0001100 | Heterochromia iridis | |
| HP:0034820 | Koeppe nodules | |
| Occasional (29-5%) | ||
| HP:0030534 | Abnormal best corrected visual acuity test | |
| HP:0007777 | Chorioretinal scar | |
| HP:0000501 | Glaucoma | |
| HP:0007906 | Ocular hypertension | |
| HP:0200026 | Ocular pain | |
| HP:0001085 | Papilledema | |
| HP:0000613 | Photophobia | |
| HP:0025337 | Red eye | |
| HP:0007663 | Reduced visual acuity | |
| Very rare (<4-1%) | ||
| HP:0009916 | Anisocoria | |
| HP:0030554 | Best corrected visual acuity 0.1 LogMAR | |
| HP:0100014 | Epiretinal membrane | |
| HP:0000543 | Optic disc pallor | |
| HP:0011958 | Retinal perforation | |
| Excluded (0%) | ||
| HP:0011484 | Posterior synechiae of the anterior chamber | |
Run the differential on the 4 obligate / very frequent terms
Annotation source: 27781126[PMID]_16458965[PMID]_17986291[PMID]_11815885[PMID]
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/263479
- This record as JSON: /data/d/263479.json