Hello and welcome to the Zero to Finals podcast. My name is Tom and in this episode I'm going to be

talking to you about primary sclerosing colangitis. And you can find written notes on this topic

at zero definals.com slash primary sclerosing colangitis or in the gastroenterology section of the zero to finals

medicine book. So let's get straight into it. Primary sclerosing colangitis is a condition where the

intra-hypatic and extra-hypatic bile ducts become inflamed and damaged, developing strictures

that obstruct the flow of bile out of the liver and into the intestines.

Sclerosis refers to stiffening and hardening of the bile ducts.

Colangitis is inflammation of the bioldux.

Chronic bile obstruction eventually leads to liver inflammation or hepatitis, fibrosis and liver cirrhosis.

The cause of primary sclerosing collerosis is unclear and it's thought to be a combination of genetic and environmental factors. There's a strong association with alterative colitis,

with about 70% of cases occurring alongside pre-existing ulcerative colitis, with about 70% of cases occurring alongside pre-existing ulcerative colitis.

Less commonly, it can be associated with Crohn's disease.

So importantly, it's often related to inflammatory bowel disease, specifically ulcerative colitis

and less commonly Crohn's disease.

The key risk factors for primary sclerosing colangitis are being male, aged 30 to 40 years,

having ulcerative colitis, and having a family history of primary sclerosing colangitis.

Next let's talk about the presentation.

Often patients are asymptomatic at diagnosis, with the problem picked up on abnormal liver

function tests done for some other reason.

However, they may present with abdominal pain,

specifically in the right upper quadrant where the liver is, pruritus or itching of the skin,

fatigue, jaundice, hepatomegaly or an enlarged liver, and they may have spleenomegaly or an enlarged spleen.

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