Primary sclerosing cholangitis: Symptoms, causes, diagnosis

Primary sclerosing cholangitis is a rare disease that mainly affects bile ducts inside and outside the liver. It can also occur in the ducts of the gallbladder and pancreas.

In cases of primary sclerosing cholangitis (PSC), the walls of the bile ducts become inflamed, leading to scarring and hardening. These changes cause the ducts to narrow, making it difficult for bile to drain through them.

As a result, bile accumulates in the liver, where it damages liver cells. Eventually, bile seeps into the bloodstream.

After sustaining long-term damage, the liver develops cirrhosis, which is a hardening, or fibrosis. It can no longer function properly.

PSC is closely linked to chronic ulcerative colitis, Crohn’s disease, and colon cancer, but it can also occur alone.

The disease is more likely to develop in males, who make up 70 percent of people with PSC. Symptoms appear, on average, at the age of 40 in males and 45 in females.

What is primary sclerosing cholangitis?

Tiredness is a symptom
Tiredness can be a symptom of primary sclerosing cholangitis.

The liver produces bile, a greenish-brown fluid that is necessary for digestion.

Liver cells excrete bile into tiny tubes within the liver called bile ducts. These tubes come together like the veins of a leaf.

These small tubes drain bile into a common duct, a larger tube that leads into the intestine. There, the bile assists digestion and gives stool its brown color.

In a person with PSC, inflammation and scarring cause the bile ducts to become blocked.

As a result, bile collects in the liver. Over time, it damages liver cells and causes fibrosis, or cirrhosis, which is scarring of the liver.

As cirrhosis progresses, more scar tissue builds up, and the liver gradually becomes unable to function. The scar tissue may prevent the bile ducts from draining, which can lead to infection in the ducts.

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Causes

Scientists do not know exactly what causes PSC. According to Genetics Home Reference, a combination of environmental and genetic factors likely play a role.

person with stomach pain
Primary sclerosing cholangitis often occurs alongside Crohn’s disease and other types of inflammatory bowel disease.

Some researchers have suggested that PSC could be an autoimmune condition. These conditions arise when the functioning of the immune system changes.

Usually, the immune system protects the body from infections caused by foreign invaders such as bacteria and viruses.

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