Primary sclerosing cholangitis (PSC)
A chronic disease in which the bile ducts inside and outside the liver become inflamed and scarred, narrowing and blocking the flow of bile. It develops slowly, is strongly linked to inflammatory bowel disease, and can progress over years to cirrhosis; it also raises the risk of bile-duct cancer.
What it is
Primary sclerosing cholangitis is a long-term condition in which the bile ducts — the tubes that carry bile from the liver to the gut — become progressively inflamed, scarred and narrowed. The scarring creates a characteristic pattern of strictures (narrowings) and dilated segments, obstructing bile flow so that bile backs up in the liver (cholestasis). The cause is not fully understood but is thought to be immune-mediated, and it is very strongly associated with inflammatory bowel disease, especially ulcerative colitis — most people with PSC have or will develop bowel inflammation. It usually affects younger and middle-aged adults, and men more than women. Many people have no symptoms at first and it is picked up from abnormal liver tests. Over time it can cause fatigue, itching, right-upper-abdominal discomfort, and episodes of bile-duct infection (cholangitis) with fever and jaundice. Diagnosis rests on a cholestatic pattern of liver enzymes (a raised alkaline phosphatase and GGT) together with the typical bile-duct appearance on MRI (MRCP). Over years it can progress to cirrhosis and liver failure, and it carries an increased risk of cancer of the bile ducts, gallbladder and, via the associated colitis, the bowel. There is no cure that reverses it; care focuses on managing symptoms and complications, surveillance for cancer, and, for advanced disease, liver transplantation.
Key lab markers
- Alkaline phosphatase (ALP) — typically raised; the hallmark cholestatic enzyme.
- GGT — raised, confirming the ALP is from the liver/bile ducts.
- Bilirubin — can be normal early, rising with more advanced disease or a dominant stricture.
- ALT and AST — often mildly raised.
- IgG4 — checked to distinguish the IgG4-related form, which is treated differently.
- Albumin and INR — assess liver function as the disease advances.
Symptoms
- Often none at first (found on abnormal liver tests)
- Fatigue
- Itching (pruritus)
- Discomfort in the right upper abdomen
- Jaundice (yellowing), often intermittent
- Episodes of fever, chills and jaundice from bile-duct infection (cholangitis)
- Weight loss in advanced disease
- Symptoms of associated inflammatory bowel disease (diarrhoea, blood in stool)
Related lab panels
When to discuss with a doctor
Primary sclerosing cholangitis is a specialist-managed condition. It should be considered when there is a persistent cholestatic pattern of liver tests (a raised alkaline phosphatase and GGT), particularly in someone with inflammatory bowel disease, and is confirmed with bile-duct imaging (MRCP). Because it can progress silently and raises the risk of bile-duct and bowel cancer, it needs specialist follow-up with regular monitoring and cancer surveillance, even when symptoms are mild. Fever with jaundice suggests a bile-duct infection (cholangitis) and needs urgent care. Mediora.AI can surface the cholestatic liver-enzyme pattern that should prompt evaluation for PSC, but the diagnosis, monitoring and treatment — including decisions about transplantation — belong with a liver specialist.