Synopsis
Diagnose primary sclerosing cholangitis non-invasively, recognise bacterial or malignant obstruction and coordinate inflammatory bowel, cancer and transplant surveillance.
- PSC is a chronic fibroinflammatory cholangiopathy producing multifocal stricturing and segmental dilatation of intrahepatic or extrahepatic bile ducts.
- Most people have or later develop inflammatory bowel disease, commonly a colitis phenotype whose activity can be clinically quiet despite increased colorectal cancer risk.
- Magnetic resonance cholangiopancreatography is the preferred diagnostic duct map; ERCP is reserved for tissue sampling or therapy after expert review.
Key red flags
Fever or rigors with pain, jaundice, hypotension or confusion implies infected obstruction and needs immediate antibiotics and drainage assessment.
Investigation priorities
Characterise cholestasis and recognise deterioration.
Management branches
Persistent cholestasis or an IBD-associated liver-test abnormality raises a cholangiopathy.
- Review medicines, infection, surgery and IBD history, measure IgG4 and obtain ultrasound to exclude an obvious mechanical or secondary cause.
- Perform high-quality MRCP and have a hepatobiliary radiologist assess distribution, strictures, gallbladder and any malignant feature.
Key medicines
Intravenous antibacterial therapy for acute cholangitisStart the hospital's current biliary-sepsis regimen immediately after cultures when feasible, adjusting for allergy, renal function, previous cultures and healthcare exposure; review promptly after drainage and microbiology results.
Cholestyramine for pruritusUse the licensed BNF regimen as a specialist or primary-care supervised trial, beginning at a tolerable dose and taking it at least four to six hours apart from essential oral medicines.