Autoimmune hepatitis type 1
Synonyms: AIH type 1
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A form of autoimmune hepatitis characterized by clinical presentation as cryptogenic hepatitis, interface hepatitis on histological examination, elevated serum aminotransferase levels, hypergammaglobulinemia/elevated immunoglobulin G, and presence of circulating autoantibodies, specifically antinuclear antibodies (ANA), anti-smooth muscle antibodies (anti-SMA), and/or anti-soluble liver antigen/liver pancreas antigen antibodies (anti-SLA/LP). The disease predominantly develops at a post-pubertal age and most commonly takes a chronic course, although acute or acute severe presentation may also be observed. Typical concurrent autoimmune diseases are autoimmune thyroiditis and rheumatic diseases.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Autoimmune hepatitis type 1 | AIH type 1 |
| fr | Hépatite auto-immune type 1 | HAI type 1 |
| de | Hepatitis, autoimmune, Typ 1 | AIH Typ 1 |
| es | Hepatitis autoinmune tipo 1 | HAI tipo 1 |
| it | Epatite autoimmune tipo 1 | EA tipo 1 |
| nl | Auto-immune hepatitis type 1 | = en |
| pt | Hepatite autoimune tipo 1 | |
| pl | Autoimmunizacyjne zapalenie wątroby typu 1 | = en |
| cs | Autoimunitní hepatitida typu 1 | = en |
| uk | Аутоімунний гепатит, тип 1 | AIH тип 1 |
| zh | 自身免疫性肝炎1型 | = en |
| tr | Otoimmün hepatit tip 1 | AIH tip 1 |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K75.4 | NTBT | attributed | validated |
| ICD-11 | DB96.0 | NTBT | index | validated |
| MONDO | 0005543 | E | validated | |
| UMLS | C4303164 | 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:2137Autoimmune hepatitis
rare hepatic diseases; rare transplant-related disorders
No children.
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/563576
- This record as JSON: /data/d/563576.json