Primary Sclerosing Cholangitis

Primary sclerosing cholangitis (PSC) is a disease of the bile ducts. It causes chronic inflammation in your bile ducts (cholangitis), which eventually causes scarring (sclerosis). Scar tissue in your bile ducts causes them to narrow, which restricts the flow of bile through the ducts (biliary stricture). When bile can’t flow, it backs up into your liver, where it came from. This does progressive damage to your liver.

Is primary sclerosing cholangitis serious?

Yes. While you might not have symptoms at first, PSC is a progressive disease that gets worse over time. When bile stalls in your bile ducts (cholestasis), it can begin to leak bile toxins into your bloodstream, which makes you sick. It also does progressive damage to your liver. Within 10 to 15 years, this can lead to liver failure. You can’t live without a liver. While there are temporizing treatments, there is currently no cure for PSC, except a liver transplant.

What is the difference between primary sclerosing cholangitis and secondary sclerosing cholangitis?

“Primary” means that this disease is the original cause of inflammation and scarring of your bile ducts. There’s no other cause but the disease. In secondary sclerosing cholangitis, inflammation (cholangitis) and scarring (sclerosis) of your bile ducts are secondary effects caused by something else. Some causes of secondary sclerosing cholangitis include:

• Bile duct injury (for example, during surgery).
• Infections of the bile ducts.
• Chemotherapy.
• Gallstones in your bile ducts.
• Recurring pancreatitis.

What is the difference between primary biliary cirrhosis and primary sclerosing cholangitis?

Primary biliary cholangitis (PBC) is the current name for what was previously called primary biliary cirrhosis.

PBC and PSC are both progressive bile duct-related liver diseases, with similar symptoms and effects. Both diseases cause the gradual deterioration of your bile ducts. Destruction and scarring in your bile ducts cause them to narrow and the flow of bile to stall. The bile begins to back up into your liver, where it causes further damage. This leads to scarring and eventually, cirrhosis of the liver.

Here are the primary differences:

• PBC only affects the bile ducts within your liver (intrahepatic), while PSC can affect the bile ducts inside or outside of your liver (extrahepatic).
• PBC is more predominant in people assigned female at birth, by a ratio of 10:1. PSC is more predominant in people assigned male at birth, by a ratio of 2:1.
• PSC is highly associated with preexisting IBD (80%), while PBC is not.
• PSC is associated with an increased risk of developing bile duct cancer, while PBC is not.
• PBC treatment involves using ursodeoxycholic acid (UDCA) to slow its progression. PSC currently has no medical treatment.

How common is this condition?

PSC is rare. It’s estimated to occur in about 1 in 10,000 people worldwide.

Who does it affect?

PSC occurs in people assigned male at birth twice as often as in people assigned female at birth. It’s most often diagnosed around the age of 40. About 80% of people with PSC also have inflammatory bowel disease, most often ulcerative colitis. It’s more likely to occur in people with a family history of the disease.