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.
- A normal MRCP does not exclude small-duct PSC when cholestasis and compatible biopsy findings persist.
- IgG4-related sclerosing cholangitis, secondary obstruction, ischaemic injury, infection, surgery and drug injury must be excluded before calling the process primary.
- No medicine has convincingly altered the natural history for all patients, and routine high-dose ursodeoxycholic acid is not recommended.
- New jaundice or deterioration can reflect stones, bacterial cholangitis, a treatable relevant stricture or cholangiocarcinoma rather than inevitable disease progression.
- CA19-9 has insufficient diagnostic accuracy for screening alone; it can support but never establish cholangiocarcinoma, and some people do not express the antigen.
- PSC with colitis requires high-quality regular colonoscopic surveillance from diagnosis under the current BSG IBD pathway, even when bowel symptoms are minimal.
- Early transplant referral is appropriate for decompensation, recurrent bacterial cholangitis, intractable pruritus or selected cancer pathways, not only a high numerical score.
Key red flags
Fever or rigors with pain, jaundice, hypotension or confusion implies infected obstruction and needs immediate antibiotics and drainage assessment.
New persistent jaundice, weight loss, a changing stricture, gallbladder lesion or unexpectedly progressive cholestasis requires urgent HPB multidisciplinary investigation.
Thrombocytopenia, splenomegaly, ascites, variceal bleeding, encephalopathy or sarcopenia indicates advanced disease and prompts transplant-level review.
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.
- Reserve ERCP for a defined sampling or therapeutic problem, and use liver biopsy when ducts are normal but small-duct PSC or overlap remains plausible.
- At diagnosis stage fibrosis, investigate colitis with ileocolonoscopy and create a documented liver, biliary and bowel surveillance plan.
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.Antibiotics cannot compensate for an undrained infected system. Review Clostridioides difficile risk, interactions and local resistance, and avoid indefinite prophylaxis without a specialist recurrent-cholangitis plan.
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.Constipation, bloating and poor palatability are common. It can reduce absorption of anticoagulants, vitamins and other treatment; exclude a new obstructing stricture before attributing worsening itch to chronic disease.