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Start free with EleplanPrimary biliary cholangitis
ORPHA:186Disease
Also called Hanot syndrome · PBC · Primary biliary cirrhosis
What it is
A rare autoimmune cholestatic liver disease characterized by autoimmune mediated damage of small intrahepatic bile ducts leading to cholestasis, fibrosis, and potential cirrhosis.
Key facts
- Prevalence
- 1-5 / 10 000
- Age of onset
- Adolescent, Adult, Elderly
- Inheritance
- Multigenic/multifactorial, Unknown
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Very common80–99%
6Common30–79%
20- Abnormal circulating lipid concentration
- Abnormality of the intrahepatic bile duct
- Abnormality of the thyroid gland
- Antinuclear antibody positivity
- Autoimmunity
- Elevated circulating alkaline phosphatase concentrationDiagnostic criterion
- Elevated gamma-glutamyltransferase level
- Hepatic failure
- Hepatic fibrosis
- Hepatocellular carcinoma
- Hepatomegaly
- Hypercholesterolemia
- Increased circulating IgM level
- Jaundice
- Onychomycosis
- Orthostatic hypotension
- Portal hypertension
- Pruritus
- Recurrent fungal infections
- Xanthelasma
Sometimes5–29%
14- Abdominal distention
- Ascites
- Celiac disease
- Esophageal varix
- Excessive daytime somnolence
- Fatigue
- Hepatic encephalopathy
- Hepatitis
and 6 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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