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primary biliary cholangitis

MONDO:0005388Mondo

Findings

No curated finding names primary biliary cholangitis yet. Everything below is reference data from Mondo, HPO and GenCC, not evidence about what affects it.

Primary biliary cholangitis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure.

Definition from the Mondo Disease Ontology (MONDO:0005388), read 2026-09-29. CC BY 4.0.

Features

41 features

What the disease is reported to present with, and how often among people who have it — never how often a feature means the disease. HPO, annotations 2026-09-02.

Antimitochondrial antibody positivityHPOHP:0030167
- Very frequent (80% to 99% of cases)
Biliary cirrhosisHPOHP:0002613
- Very frequent (80% to 99% of cases)
CirrhosisHPOHP:0001394
- Very frequent (80% to 99% of cases)
Conjugated hyperbilirubinemiaHPOHP:0002908
- Very frequent (80% to 99% of cases)
Dermatographic urticariaHPOHP:0011971
- Very frequent (80% to 99% of cases)
Hyperpigmentation of the skinHPOHP:0000953
- Very frequent (80% to 99% of cases)
Abnormal circulating lipid concentrationHPOHP:0003119
- Frequent (30% to 79% of cases)
Abnormal intrahepatic bile duct morphologyHPOHP:0011040
- Frequent (30% to 79% of cases)
Abnormality of the thyroid glandHPOHP:0000820
- Frequent (30% to 79% of cases)
Antinuclear antibody positivityHPOHP:0003493
- Frequent (30% to 79% of cases)
AutoimmunityHPOHP:0002960
- Frequent (30% to 79% of cases)
Elevated circulating alkaline phosphatase concentrationHPOHP:0003155
- Frequent (30% to 79% of cases)

Show the remaining 29

Elevated gamma-glutamyltransferase levelHPOHP:0030948
- Frequent (30% to 79% of cases)
Hepatic failureHPOHP:0001399
- Frequent (30% to 79% of cases)
Hepatic fibrosisHPOHP:0001395
- Frequent (30% to 79% of cases)
Hepatocellular carcinomaHPOHP:0001402
- Frequent (30% to 79% of cases)
HepatomegalyHPOHP:0002240
- Frequent (30% to 79% of cases)
HypercholesterolemiaHPOHP:0003124
- Frequent (30% to 79% of cases)

Where it sits

A kind of: biliary tract disorder
cirrhosis, familial
Narrower terms (5): primary biliary cholangitis 1
primary biliary cholangitis 2
primary biliary cholangitis 3
primary biliary cholangitis 4
primary biliary cholangitis 5

Other names

6 names

Resolves to: primary biliary cholangitis

Also called: chronic non-suppurative destructive cholangitischronic nonsuppurative destructive cholangitisHanot syndromePBCprimary Bilary cirrhosis (PBC)primary biliary cirrhosis