IgG4-related sclerosing cholangitis
- Type
- Clinical subtype
- Group
- Subtype of disorder
- Inheritance
- Not applicable
- Status
- active
A rare systemic autoimmune disease characterized by cholestasis and diffuse cholangiographic abnormalities with circular and symmetrical bile duct wall thickening, and elevated serum IgG4 levels. Characteristic histopathological findings include dense infiltration of IgG4-positive plasma cells and extensive fibrosis in the bile duct wall. A marked response to steroid therapy is typical. Patients present with jaundice, cholangitis, pruritis, and sometimes associated findings of autoimmune pancreatitis, sialadenitis, and retroperitoneal fibrosis.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | IgG4-related sclerosing cholangitis | |
| fr | Cholangite sclérosante à IgG4 | |
| de | IgG4-assoziierte sklerosierende Cholangitis | |
| es | Colangitis esclerosante asociada a IgG4 | |
| it | Colangite sclerosante IgG4-correlata | |
| nl | IgG4-gerelateerde scleroserende cholangitis | |
| pt | Colangite esclerosante IgG4-relacionada | |
| pl | Twardniające zapalenie dróg żółciowych związane z IgG4 | |
| cs | IgG4-vázaná sklerotizující cholangitida | |
| uk | IgG4-асоційований склерозуючий холангіт | |
| zh | IgG4相关硬化性胆管炎 | |
| tr | IgG4-ilişkili sklerozan kolanjit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K83.0 | NTBT | attributed | validated |
| ICD-11 | 4A43.0 | NTBT | index | validated |
| MONDO | 0018645 | E | validated | |
| UMLS | C4302109 | 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 (2):
- ORPHA:596448IgG4-related systemic disease
rare systemic and rheumatological diseases - ORPHA:447771Sclerosing cholangitis
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/447764
- This record as JSON: /data/d/447764.json