Primary Biliary Cholangitis

ICD-10: DB95.0MeSH: D015209ORPHA: 186

Overview

A chronic autoimmune liver disease characterized by progressive destruction of intrahepatic bile ducts leading to cholestasis and cirrhosis. Predominantly affects middle-aged women and has a prevalence of approximately 1 in 3,000-4,000 individuals.

Available Treatments (0)

No treatments linked yet

Clinical trials or compassionate use may be available — consult a specialist.

Clinical Presentation

Signs and symptoms associated with Primary Biliary Cholangitis, sourced from HPO and Orphanet clinical annotations.

Hyperpigmentation of the skinCirrhosisBiliary cirrhosisConjugated hyperbilirubinemiaDermatographic urticariaAntimitochondrial antibody positivityAbnormality of the thyroid glandJaundicePruritusXanthelasmaOrthostatic hypotensionHepatic fibrosisHepatic failureHepatocellular carcinomaPortal hypertensionHepatomegalyRecurrent fungal infectionsAutoimmunityAbnormal circulating lipid concentrationHypercholesterolemiaElevated circulating alkaline phosphatase concentrationAntinuclear antibody positivityIncreased circulating IgM levelAbnormal intrahepatic bile duct morphologyOnychomycosisElevated gamma-glutamyltransferase levelOsteoporosisExcessive daytime somnolenceAscitesSplenomegalyEsophageal varixSleep disturbanceHepatic encephalopathySteatorrheaCeliac diseaseHypoalbuminemiaIncreased circulating IgA concentrationAbdominal distentionHepatitisFatigueGastrointestinal inflammation

Classification & Codes

ICD-10 Code

DB95.0

MeSH Code

D015209

Orphanet Code

ORPHA:186
Primary Biliary Cholangitis
ICD-10DB95.0
MeSHD015209
OrphanetORPHA:186
Treatments0 drug(s)
Symptoms on record41 signs
Statuspublished
Factual Authority
Last Updated3/21/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO