Primary Sclerosing Cholangitis

ICD-10: K83.01MeSH: D015209ORPHA: 171

Contents

1. Overview
2. Available Treatments
3. Clinical Presentation
4. Classification & Codes

Overview

Primary sclerosing cholangitis is a chronic cholestatic liver disease characterized by inflammation and fibrosis of intrahepatic and extrahepatic bile ducts, leading to biliary cirrhosis. Approximately 60-80% of patients have concurrent inflammatory bowel disease, particularly ulcerative colitis. The condition carries increased risk for cholangiocarcinoma (10-15% lifetime risk). Prevalence is estimated at 6-16 per 100,000 in Northern European populations.

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 Sclerosing Cholangitis, sourced from HPO and Orphanet clinical annotations.

CholestasisAutoimmunityAbnormal biliary tract morphologyCirrhosisHepatic fibrosisPortal hypertensionHepatosplenomegalyAscitesSplenomegalyWeight lossFeverHepatomegalyElevated circulating hepatic transaminase concentrationElevated alkaline phosphatase of hepatic originSpider hemangiomaAbnormal large intestine physiologyDilated superficial abdominal veinsUlcerative colitisPalmar telangiectasiaRenal insufficiencyDepressionOsteopeniaOsteoporosisJaundicePruritusCholelithiasisHepatocellular carcinomaCongestive heart failurePancreatitisAbdominal painPleural effusionCeliac diseaseHypoalbuminemiaPolyclonal elevation of IgMGeneralized amyotrophyReduced circulating vitamin A concentrationProlonged prothrombin timeDecreased circulating vitamin K concentrationHepatitisFatigueCholangiocarcinomaAdenocarcinoma of the large intestineDecreased circulating vitamin D concentrationDecreased circulating vitamin E concentrationChronic hepatic failureThyroiditisType I diabetes mellitusUveitisEncephalopathyAcute hepatic failureNeoplasm of the gallbladder

Classification & Codes

ICD-10 Code

K83.01

MeSH Code

D015209

Orphanet Code

ORPHA:171

Primary Sclerosing Cholangitis

ICD-10	`K83.01`
MeSH	`D015209`
Orphanet	`ORPHA:171`
Treatments	0 drug(s)
Symptoms on record	51 signs
Status	published

Factual Authority

Last Updated3/21/2026

Clinical DataHPO · Orphanet

Drug DataFDA · EMA · CDSCO