FFinalistSouthampton · 2027
Gastroenterology
AKT · Gastroenterology/Liverlow yield

Primary sclerosing cholangitis

Chronic fibro-inflammatory stricturing of intra/extra-hepatic bile ducts (UC-associated)

Overview

A chronic cholestatic disease of progressive inflammation, fibrosis and STRICTURING of the intra- and extra-hepatic bile ducts. Classically a MAN with ULCERATIVE COLITIS; cholestatic LFTs with a characteristic BEADED (multifocal stricture) appearance on MRCP. Raised cholangiocarcinoma and colorectal cancer risk. No effective medical therapy slows it; manage strictures and complications; transplant for end-stage disease.

Recognise

  • Often a MAN with inflammatory bowel disease (especially ULCERATIVE COLITIS); fatigue, pruritus, RUQ pain, intermittent jaundice/cholangitis
  • Cholestatic LFTs (ALP up); MRCP: multifocal strictures + dilatation = BEADED ducts; p-ANCA often positive
  • Markedly raised cholangiocarcinoma risk (and colorectal cancer with UC) - surveillance

Red flags

  • Cholangiocarcinoma (rising CA19-9/new dominant stricture); recurrent bacterial cholangitis
  • Colorectal cancer (UC + PSC - higher risk, surveillance)

Differentials & how to tell them apart

Primary biliary cholangitiswomen + AMA + small intrahepatic ducts - PSC is men + UC + beaded large ducts + ANCA
Secondary sclerosing cholangitisprior surgery/stones/ischaemia/IgG4 - exclude
Cholangiocarcinomaa dominant stricture - the feared complication of PSC

Investigations

Cholestatic LFTs; MRCP (BEADED ducts - diagnostic); p-ANCA; colonoscopy (associated IBD); CA19-9 + surveillance for cholangiocarcinoma; biopsy (onion-skin fibrosis) if small-duct.

Management

Manage strictures/complications + surveillance; transplant for end-stage disease

  1. 1Confirm with MRCP (beaded ducts); colonoscopy for associated IBD. No drug clearly halts it; ERCP dilates/stents dominant strictures, antibiotics treat cholangitis.Gate: Surveil for CHOLANGIOCARCINOMA (a new dominant stricture/rising CA19-9) and for COLORECTAL CANCER (UC + PSC carries higher risk - colonoscopic surveillance); PSC (men/UC/beaded/ANCA) is distinct from PBC (women/AMA)
  2. 2Transplant for end-stage disease (recurrence possible); manage cholangiocarcinoma; fat-soluble vitamins/bone protection; cholestyramine for itch.
No proven disease-modifying drug — ursodeoxycholic acid may improve LFTs but does not clearly change outcome
ERCP for dominant strictures; antibiotics for cholangitis — manage complications
Liver transplant — for end-stage disease (can recur)

Key points

Man + UC + cholestatic LFTs + beaded ducts on MRCP + p-ANCA = PSC. Watch hard for cholangiocarcinoma and colorectal cancer. PBC is the women/AMA mirror image.

Monitor & prognosis

LFTs/CA19-9; MRCP; colonoscopic surveillance.

Progressive; cancer risk; transplant for end-stage.

Source: EASL; BSG