Autoimmune polyendocrinopathy
Synonyms: APS · Autoimmune polyglandular syndrome
- Type
- Clinical group
- Group
- Group of disorders
- Status
- active
A group of rare endocrine diseases characterized by autoimmune activity against more than one endocrine organ, with possible additional involvement of non-endocrine organs. Autoimmunity is typically directed against different target antigens in different tissues. The two more common autoimmune polyendocrine syndromes (APS), APS type 1 and type 2, have a strong genetic background and have Addison's disease as a major feature. The group furthermore includes APS type 3 and type 4.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Autoimmune polyendocrinopathy | APS · Autoimmune polyglandular syndrome |
| fr | Polyendocrinopathie auto-immune | APS |
| de | Polyendokrinopathie, autoimmune | Autoimmun-polyglanduläres Syndrom |
| es | Poliendocrinopatía autoinmune | SPA · Síndrome poliglandular autoinmune |
| it | Poliendocrinopatia autoimmune | APS · Sindrome polighiandolare autoimmune |
| nl | Auto-immune polyendocrinopathie | APS · Auto-immuun polyglandulair syndroom |
| pt | Poliendocrinopatia autoimune | APS · Síndrome poliglandular autoimune |
| pl | Poliendokrynopatia autoimmunologiczna | APS · Autoimmunologiczny zespół wielogruczołowy |
| cs | Autoimunitní polyendokrinopatie | APS · Autoimunitní polyglandulární syndrom |
| uk | Аутоімунна поліендокринопатія | APS · Аутоімунний полігландулярний синдром |
| zh | 自身免疫性多内分泌病 | 自身免疫性多腺体综合征 |
| tr | Otoimmün poliendokrinopati | APS · Otoimmün poliglandüler sendrom |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | E31.0 | E | specific | validated |
| ICD-11 | 5B00 | E | specific | validated |
| MONDO | 0017278 | E | validated | |
| MONDO | 17278 | E | validated | |
| MeSH | D016884 | E | validated | |
| UMLS | C0085409 | 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:101956Polyendocrinopathy
rare endocrine diseases
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
0 recordsNo prevalence record in this release.
HPO phenotypes
Orphanet has no HPO annotation for this code in this release.
Sources for this card
- Orphanet page: orpha.net/en/disease/detail/282196
- This record as JSON: /data/d/282196.json