Follicular cholangitis and pancreatitis
Synonyms: Follicular pancreatocholangitis
- Type
- Disease
- Group
- Disorder
- Inheritance
- Unknown
- Age of onset
- Adult
- Status
- active
Follicular cholangitis and pancreatitis is a rare pancreatobiliary disease characterized by marked duct-centered lymphoid follicular inflammation that develops in both biliary and pancreatic ductal systems, mainly affecting the hilar bile ducts and the pancreatic head. Patients present with jaundice, abdominal pain, liver dysfunction, pruritus and/or weight loss. Histology shows lymphoplasmacytic infiltration with formation of numerous, large lymphpoid follicles around the affected bile and pancreatic ducts.
Names in Orphadata languages
12 of 12 languages| Lang | Name | Synonyms |
|---|---|---|
| en | Follicular cholangitis and pancreatitis | Follicular pancreatocholangitis |
| fr | Pancréatite et cholangite folliculaire | Pancréatocholangite folliculaire |
| de | Cholangitis und Pankreatitis, follikuläre | Pankreatocholangitis, follikuläre |
| es | Colangitis folicular y pancreatitis | Pancreatocolangitis folicular |
| it | Colangite follicare e pancreatite | Pancreatocolangite follicolare |
| nl | Folliculaire cholangitis en pancreatitis | Folliculaire pancreatocholangitis |
| pt | Colangite folicular e pancreatite | |
| pl | Pęcherzykowe zapalenie przewodów żółciowych i trzustki | = en |
| cs | Folikulární cholangitida a pankreatitida | Folikulární pankreatocholangitida |
| uk | Фолікулярний холангіт і панкреатит | Фолікулярний панкреатохолангіт |
| zh | 滤泡性胆管炎和胰腺炎 | 滤泡性胰腺胆管炎 |
| tr | Foliküler kolanjit ve pankreatit | Foliküler pankreatokolanjit |
Definition in:
Cross-references
qualifier = Orphanet mapping relation| Source | Code | Qualifier | ICD relation | Validation |
|---|---|---|---|---|
| ICD-10 | K83.0 | NTBT | attributed | validated |
| ICD-10 | K86.1 | NTBT | attributed | validated |
| ICD-11 | DC33 | NTBT | attributed | validated |
| MONDO | 0017589 | E | validated | |
| UMLS | C4749906 | 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:101941Rare biliary tract disease
rare hepatic diseases - ORPHA:101937Rare pancreatic disease
rare gastroenterological diseases
No children.
Genes
0 directOrphanet records no gene directly on this code.
Prevalence and incidence
2 records| Type | Class | Value | Area | Qualification | Validation | Source |
|---|---|---|---|---|---|---|
| Cases/families | 5 cases | Worldwide | Case(s) | Validated | 22211284[PMID] | |
| Point prevalence | <1 / 1 000 000 | — | Worldwide | Class only | Validated | 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/300552
- This record as JSON: /data/d/300552.json