Orphanet Rare Disease Ontology

Last uploaded: December 13, 2023
Preferred Name

Primary biliary cholangitis

Synonyms

PBC

Definitions

A rare autoimmune cholestatic liver disease characterized by autoimmune mediated damage of small intrahepatic bile ducts leading to cholestasis, fibrosis, and potential cirrhosis.

ID

http://www.orpha.net/ORDO/Orphanet_186

alternative_term

PBC

Primary biliary cirrhosis

Hanot syndrome

definition

A rare autoimmune cholestatic liver disease characterized by autoimmune mediated damage of small intrahepatic bile ducts leading to cholestasis, fibrosis, and potential cirrhosis.

definition_citation

Orphanet

expertlink

https://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=186

has_age_of_onset

Adolescent

Adult

Elderly

has_inheritance

Multigenic/multifactorial

Unknown

hasDbXref

OMIM:614220

OMIM:614221

UMLS:C0008312

ICD-10:K74.3

OMIM:613008

OMIM:613007

OMIM:109720

ICD-11:DB96.1

MedDRA:10080429

MeSH:D008105

label

Primary biliary cholangitis

notation

ORPHA:186

part_of

http://www.orpha.net/ORDO/Orphanet_506210

http://www.orpha.net/ORDO/Orphanet_101941

prefixIRI

ORDO:Orphanet_186

prefLabel

Primary biliary cholangitis

present_in

Iceland AND has_point_prevalence_average_value : 38.3 AND has_point_prevalence_range : 1-5 / 10 000

Spain AND has_annual_incidence_average_value : 1.7 AND has_annual_incidence_range : 1-9 / 100 000

Worldwide AND has_annual_incidence_average_value : 3.0 AND has_annual_incidence_range : 1-9 / 100 000

Denmark AND has_annual_incidence_average_value : 1.7 AND has_annual_incidence_range : 1-9 / 100 000

Spain AND has_point_prevalence_average_value : 19.5 AND has_point_prevalence_range : 1-5 / 10 000

Netherlands AND has_point_prevalence_average_value : 13.2 AND has_point_prevalence_range : 1-5 / 10 000

Brunei Darussalam AND has_annual_incidence_average_value : 1.0 AND has_annual_incidence_range : 1-9 / 100 000

United States AND has_point_prevalence_average_value : 40.2 AND has_point_prevalence_range : 1-5 / 10 000

United Kingdom AND has_point_prevalence_average_value : 25.0 AND has_point_prevalence_range : 1-5 / 10 000

Netherlands AND has_annual_incidence_average_value : 1.1 AND has_annual_incidence_range : 1-9 / 100 000

United States AND has_annual_incidence_average_value : 4.5 AND has_annual_incidence_range : 1-9 / 100 000

United Kingdom AND has_annual_incidence_average_value : 2.3 AND has_annual_incidence_range : 1-9 / 100 000

Europe AND has_annual_incidence_average_value : 2.57 AND has_annual_incidence_range : 1-9 / 100 000

Finland AND has_point_prevalence_average_value : 18.0 AND has_point_prevalence_range : 1-5 / 10 000

Finland AND has_annual_incidence_average_value : 1.7 AND has_annual_incidence_range : 1-9 / 100 000

Canada AND has_point_prevalence_average_value : 22.7 AND has_point_prevalence_range : 1-5 / 10 000

Canada AND has_annual_incidence_average_value : 3.0 AND has_annual_incidence_range : 1-9 / 100 000

China AND has_point_prevalence_average_value : 4.9 AND has_point_prevalence_range : 1-9 / 100 000

Iceland AND has_annual_incidence_average_value : 2.5 AND has_annual_incidence_range : 1-9 / 100 000

Greece AND has_point_prevalence_average_value : 36.5 AND has_point_prevalence_range : 1-5 / 10 000

Brunei Darussalam AND has_point_prevalence_average_value : 2.6 AND has_point_prevalence_range : 1-9 / 100 000

France AND has_annual_incidence_average_value : 0.9 AND has_annual_incidence_range : 1-9 / 1 000 000

Israel AND has_point_prevalence_average_value : 5.5 AND has_point_prevalence_range : 1-9 / 100 000

Greece AND has_annual_incidence_average_value : 2.1 AND has_annual_incidence_range : 1-9 / 100 000

Australia AND has_point_prevalence_average_value : 5.1 AND has_point_prevalence_range : 1-9 / 100 000

Worldwide AND has_point_prevalence_average_value : 21.05 AND has_point_prevalence_range : 1-5 / 10 000

Denmark AND has_point_prevalence_average_value : 12.0 AND has_point_prevalence_range : 1-5 / 10 000

Europe AND has_point_prevalence_average_value : 25.0 AND has_point_prevalence_range : 1-5 / 10 000

treeView

http://www.orpha.net/ORDO/Orphanet_506210

http://www.orpha.net/ORDO/Orphanet_101941

subClassOf

http://www.orpha.net/ORDO/Orphanet_377788

http://www.orpha.net/ORDO/Orphanet_557493

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