Seronegative autoimmune hepatitis
Synonyms: Autoantibody-negative autoimmune hepatitis · Seronegative AIH
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Age of onset
- Adult
- Status
- active
A form of autoimmune hepatitis characterized by the features of classic autoimmune hepatitis (i. e. clinical presentation as acute or chronic cryptogenic hepatitis, interface hepatitis on histological examination, elevated serum aspartate aminotransferase and alanine aminotransferase levels, hypergammaglobulinemia/elevated immunoglobulin G, therapeutic response to corticosteroids) in the absence of serum autoantibodies. Clinical manifestations include fatigue, malaise, arthralgia, jaundice, at later stages also signs of advanced chronic liver disease, such as spider nevi, caput medusae, splenomegaly, ascites, and palmar erythema. Presence of concurrent autoimmune diseases is frequently observed.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Seronegative autoimmune hepatitis | Autoantibody-negative autoimmune hepatitis · Seronegative AIH |
| fr | Hépatite auto-immune séronégative | HAI séronégative |
| de | Hepatitis, autoimmune seronegative | AIH, seronegative · Autoantikörper-negative autoimmune Hepatitis |
| es | Hepatitis autoinmune seronegativa | HAI seronegativa · Hepatitis autoinmune con autoanticuerpos negativos |
| it | Epatite autoimmune siero-negativa | EA siero-negativa · Epatite autoimmune negativa agli autoanticorpi |
| nl | Seronegatieve auto-immune hepatitis | Autoantilichaam-negatieve auto-immune hepatitis · Seronegatieve AIH |
| pt | Hepatite autoimune seronegativa | |
| pl | Autoimmunologiczne zapalenie wątroby seronegatywne | = en |
| cs | Séronegativní autoimunitní hepatitida | = en |
| uk | Серонегативний аутоімунний гепатит | Аутоантитілонегативний аутоімунний гепатит · Серонегативний АІГ |
| zh | 血清反应阴性自身免疫性肝炎 | 自身抗体阴性自身免疫性肝炎 |
| tr | Seronegatif otoimmün hepatit | Seronegatif AIH · Otoantikor negatif otoimmün hepatit |
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 | 0035400 | E | validated | |
| UMLS | C5680121 | 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/563589
- This record as JSON: /data/d/563589.json