Medicines for Primary sclerosing cholangitis

What it is

Primary sclerosing cholangitis (PSC) is a rare, chronic (long-term) liver condition in which the bile ducts — the tubes that carry bile from the liver to the gut — become inflamed and progressively scarred and narrowed. As the ducts narrow, bile flow is impaired, which can damage the liver over time and, in some people, lead to advanced scarring (cirrhosis) and its complications. The cause is not fully understood, but it is thought to involve the immune system, and it is strongly associated with inflammatory bowel disease (particularly ulcerative colitis) — many people with PSC also have, or develop, IBD. PSC often progresses slowly and varies greatly between people. Some have no symptoms and are found to have it when liver blood tests are abnormal; others develop symptoms such as tiredness, itching, and episodes of jaundice, tummy discomfort, or infections of the bile ducts (cholangitis). PSC also carries an increased risk of certain cancers (of the bile ducts, gallbladder, liver, and, in those with associated IBD, the bowel), which is why monitoring is important. Because it is complex and can progress, PSC is managed by liver specialists.

How it is treated

PSC is managed by a liver specialist (hepatologist), and, because there is currently no treatment that reliably stops it progressing, care focuses on monitoring the condition, managing symptoms and complications, and surveillance for the associated cancer risks. Diagnosis usually involves liver blood tests and specialised scans of the bile ducts (such as an MRCP), and assessment for associated IBD. Management includes: treating symptoms such as itching (which can be troublesome) and tiredness; promptly treating episodes of bile duct infection (cholangitis) with antibiotics; and, where a narrowed bile duct causes a significant blockage, procedures (such as an ERCP) to open it and improve bile flow. Regular monitoring tracks liver function and any progression, and surveillance is carried out for the increased cancer risks (including bowel surveillance in those with associated colitis). Managing any associated IBD is part of overall care, and nutritional and vitamin needs are addressed. For people whose liver disease becomes advanced, a liver transplant can be an effective treatment. The reassuring message is that, although PSC is a serious long-term condition without a current cure, specialist care can manage symptoms and complications, monitor for cancer risks, and, where needed, offer transplantation — and the condition varies greatly, with some people remaining relatively stable for a long time.