primary sclerosing cholangitis

Portal hypertension

As scarring stiffens the liver, blood backs up in the portal vein (portal hypertension), which can cause complications such as fluid build-up and bleeding.

Fatigue

Primary sclerosing cholangitis is a long-term liver disease, and fatigue is one of the ways it can lower quality of life for people who have it.

Pruritus

Itching, called pruritus, is a common symptom of primary sclerosing cholangitis and can greatly affect quality of life, disturbing sleep and worsening fatigue.

Adenocarcinoma of the large intestine

When primary sclerosing cholangitis occurs together with inflammatory bowel disease, colorectal cancer is more common than in inflammatory bowel disease alone.

Cholangiocarcinoma

People with primary sclerosing cholangitis have a higher risk of cholangiocarcinoma, a cancer of the bile ducts, which is one reason a narrowed bile duct is examined carefully to tell inflammation apart from cancer.

Elevated alkaline phosphatase of hepatic origin

A raised level of the liver enzyme alkaline phosphatase is a common marker of the blocked bile flow seen in primary sclerosing cholangitis, and a fall in this level can be a sign that the disease is responding.

Abdominal pain

Many people with PSC have pain in the upper right side of the abdomen, where the liver and bile ducts sit. The pain often tracks with how blocked and inflamed the bile ducts are.

Cholestasis

In primary sclerosing cholangitis, ongoing damage to the bile ducts blocks the normal flow of bile, a state called cholestasis. This buildup is a hallmark of the disease and drives many of its other features.

Jaundice

Jaundice, a yellowing of the skin and eyes, develops when blocked bile ducts let bile pigments build up in the blood. It tends to appear as the disease advances.

Abnormal biliary tract morphology

The hallmark of the disease is multiple narrowed, scarred segments of the bile ducts. On imaging such as MRCP this gives a characteristic beaded appearance that is central to the diagnosis.

Cirrhosis

Over time, the ongoing inflammation and scarring of the bile ducts in primary sclerosing cholangitis can lead to biliary cirrhosis, in which the liver becomes badly scarred and can eventually fail.

Hepatic fibrosis

PSC is driven by ongoing inflammation and scarring (fibrosis) of the bile ducts and surrounding liver. As the scarring builds up over years it can progress to cirrhosis and liver failure.

Ulcerative colitis

Primary sclerosing cholangitis is closely linked to inflammatory bowel disease, and most people with it also have bowel inflammation such as ulcerative colitis.

Dominant biliary stricture

Primary sclerosing cholangitis can produce a dominant or high-grade narrowing of a bile duct that blocks bile flow; opening it with a balloon can drain the bile, ease symptoms, and help extend survival.

Primary sclerosing cholangitis

Diagnosed using: MRCP (magnetic resonance cholangiopancreatography).

liver transplantation

There is no medicine proven to change the course of primary sclerosing cholangitis. For advanced disease, liver transplantation is the only treatment that is definitive and prolongs life.

Used to help with: Primary sclerosing cholangitis.

endoscopic balloon dilation of dominant stricture

When a single bile duct in primary sclerosing cholangitis becomes badly narrowed, doctors can widen it during an endoscopic procedure (ERCP) using a balloon to restore bile drainage and relieve symptoms.

Used to help with: Primary sclerosing cholangitis.

inflammatory bowel disease association

Most people with primary sclerosing cholangitis also have inflammatory bowel disease. This combination behaves as a distinct pattern and raises the risk of colorectal cancer and of needing a liver transplant.

Described as modulating: Primary sclerosing cholangitis.