Immune-mediated scleritis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Not applicable
- Age of onset
- All ages
- Status
- active
A rare scleritis characterized by severe ocular inflammation of sclera associated with an underlying systemic inflammatory condition, most often rheumatoid arthritis, granulomatosis with polyangiitis, but also seronegative spondyloarthropathies, vasculitides and systemic lupus erythematosus. Ocular presentation is a tender or painful, violet-blueish eye, with injection of deep scleral vessels. It can be unilateral or bilateral. Immune-mediated scleritis is more often granulomatous and/or necrotizing. Systemic clinical signs depend on the underlying disease.
Names in Orphadata languages
10 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Immune-mediated scleritis | |
| fr | Sclérite auto-immune | |
| de | Skleritis, immunvermittelte | |
| es | Escleritis inmunomediada | |
| it | Sclerite immuno-mediata | |
| nl | Immuungemedieerde scleritis | |
| pt | Esclerite imunomediada | |
| pl | Zapalenie twardówki pochodzenia immunologicznego | |
| cs | Imunitně podmíněná skleritida | |
| uk | Імуноопосередкований склероз |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | H15.0 | NTBT | specific | validated |
| ICD-11 | 9B51 | NTBT | attributed | validated |
| MONDO | 0958266 | E | validated | |
| MONDO | 958266 | E | validated | |
| UMLS | C5578029 | E | validated | |
| MedDRA | 10087281 | 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:648559Rare scleritis
rare ophthalmic disorders
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Point prevalence | Unknown | — | Worldwide | Class only | Validated | ORPHANET |
| Annual incidence | 1-9 / 100 000 | 4 / 100 000 | Worldwide | Value and class | Validated | 32504648[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.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/648681
- This record as JSON: /data/d/648681.json