Primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, and primary sclerosing cholangitis (PSC) both involve the breakdown of your bile ducts. Both conditions are also thought to result from autoimmune reactions.

Your bile ducts carry bile from your liver to your gallbladder and small intestines. Bile helps your body break down fats in your food.

Damage to your bile ducts can lead to the buildup of bile inside your liver, as well as complications including:

The primary difference between PBC and PSC is that PBC primarily involves damage to small ducts inside your liver, whereas PSC involves damage to medium and large ducts inside and outside your liver.

This article will look at the similarities and differences of PBC and PSC.

How common are PBC and PSC?

PBC is estimated to affect about 58 women and 15 men out of every 100,000 people in the United States. The average age at the time of diagnosis is about 60 years.

PSC is estimated to affect 5–16 people per 100,000. It’s most common in people between ages 30 and 40 years, and it develops about twice as often in males. About half of people are over age 41 years when they receive a diagnosis.

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Symptoms are similar between PBC and PSC.

Primary biliary cirrhosis symptoms

The most common symptoms of PBC are:

You might also develop:

Later symptoms might include:

Primary sclerosing cholangitis symptoms

Symptoms of PSC can include:

PSC can also lead to a bile duct infection, which might cause:

  • chills
  • fever
  • worsening jaundice
  • abdominal pain
  • symptoms of cirrhosis

Experts theorize that PBC and PSC both result from autoimmune reactions. A combination of genetics and environmental factors likely play a role in their development.

Primary biliary cirrhosis causes

People with parents or siblings with PBC have a 100 times higher risk of developing it than a person without an affected relative.

People with PBC-associated genes might be more likely to develop PBC if they’re exposed to certain environmental factors, such as:

  • infections
  • cigarette smoke
  • certain chemicals, such as those found in some:
    • hair dyes
    • nail polish
    • toxic waste

Primary sclerosing cholangitis causes

The development of PSC is not well understood. It may be caused by a combination of:

PSC is highly associated with inflammatory bowel disease (IBD). About 60–80% of people with PSC have irritable bowel disorder (IBD), according to 2023 research, and in about 80% of cases, they have ulcerative colitis.

It’s important to get medical attention if you think you might have an issue with your liver. Signs of a liver issue may include:

  • jaundice
  • intensely itchy skin
  • swelling in your abdomen
Medical emergency

It’s critical to call emergency medical services or go to the nearest emergency room if you or somebody else also develops any of the following symptoms:

Doctors use a variety of tests to diagnose PBC and PSC, including:

Here are the treatment options for PBC and PSC.

Primary biliary cirrhosis treatment

Medications are the primary treatments for PBC. Doctors often prescribe ursodiol to slow the progression of liver damage. If this medication isn’t effective, your doctor may prescribe obeticholic acid.

They may also suggest:

  • other medications may help treat symptoms like itchy skin
  • medications for osteoporosis
  • fat-soluble vitamin supplements
  • frequent eye and dental exams
  • statins for high cholesterol
  • additional treatment for liver complications

Primary sclerosing cholangitis treatment

No effective treatment has been developed for PSC. Doctors may use ERCP or stents to open narrowed bile ducts.

Treatments that have not been found effective or have conflicting evidence of effectiveness include:

  • ursodeoxycholic acid
  • budesonide
  • tacrolimus
  • methotrexate
  • penicillamine
  • antitumor necrosis factor antibodies
  • prednisolone
  • colchicine
  • azathioprine
  • mycophenolate mofetil

Doctors haven’t established a way to prevent PBC or PSC. You may be able to minimize liver damage by:

  • quitting smoking (which can be difficult if you smoke, but a doctor can help build a cessation plan that works for you)
  • following your doctor’s instructions
  • avoiding alcohol
  • having regular checkups
  • speaking with your doctor before taking new medications or supplements
  • maintaining or achieving a healthy body weight for you
  • avoiding illegal drugs

PBC and PSC both involve damage to your bile ducts. PSC primarily causes damage to the medium and large bile ducts inside and outside your liver. PBC is characterized by damage to the small bile ducts inside your liver.

Researchers aren’t entirely sure what causes either condition, but they may result from autoimmune reactions. A combination of genetics and environmental factors likely play a role in their development.