primary sclerosing cholangitis

MeSH: C536419ORPHA: 171

Overview

sclerosing cholangitis characterized by fibroobliterative inflammation of the biliary tract, leading to cirrhosis and portal hypertension

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

MeSH Code

C536419

Orphanet Code

ORPHA:171
primary sclerosing cholangitis
MeSHC536419
OrphanetORPHA:171
Treatments0 drug(s)
Symptoms on record51 signs
Statuspublished
Factual Authority
Last Updated3/20/2026
Clinical DataHPO · Orphanet
Drug DataFDA · EMA · CDSCO
primary sclerosing cholangitis | OrphanDrug