Primary biliary cholangitis (PBC, formerly known as primary biliary cirrhosis) and primary sclerosing cholangitis (PSC) may sound similar, but there are some key differences between these two liver diseases.

PBC and PSC are primary diseases, meaning they develop independently without a separate known cause or trigger. They’re also autoimmune diseases, meaning they’re caused by the body’s immune system mistakenly attacking its own tissues. Additionally, both conditions cause inflamed bile ducts in the liver that disrupt the organ’s normal functioning. However, the extent of damage they cause, who they affect, and their available treatments aren’t quite the same.

PBC and PSC are cholestatic liver diseases — they affect the flow of bile through your liver. Bile is the fluid that helps the body digest fats and remove toxins. It’s made in the liver and stored in the gallbladder. Bile ducts are tubes that help bile flow from the liver to the gallbladder and small intestine. Damage to these passageways can cause symptoms throughout the body.

Here’s how to make sense of PBC and PSC, two related but distinct diagnoses.

1. The Location of Each Liver Disease

PBC attacks the small bile ducts within the liver. PSC is more widespread and may extend to bile ducts outside the liver. The terms for these bile ducts are intrahepatic (inside the liver) and extrahepatic (outside the liver). Intrahepatic bile ducts are tiny tubes that can only be seen with a microscope, whereas the extrahepatic bile ducts are large enough to show up in imaging tests.

2. Risk Factors for PBC and PSC

A person’s sex plays a big role in who gets PBC and PSC. According to Cleveland Clinic, females have a 10 times higher chance of developing PBC than males whereas PSC affects twice as many males as females. PSC is also strongly associated with inflammatory bowel disease (IBD), mainly ulcerative colitis.

PBC usually begins later than PSC, at an average age of 55. PSC is typically diagnosed between ages 30 and 40. The rates of both PSC and PBC are slightly higher in some parts of the world, including Northern Europe. Overall, both conditions appear to be on the rise, but researchers aren’t sure if that’s because more people are getting the disease or if it’s due to better practice guidelines for diagnosing them.

Symptoms for either condition can overlap and include:

• Abdominal pain
• Jaundice
• Pruritus (itching)
• Fatigue
• High cholesterol

Your doctor can run various imaging and blood tests, and they may need to do a liver biopsy — removal of some liver tissue for examination under a microscope — to figure out exactly what’s going on.

Many people don’t suspect PBC or PSC from the symptoms they experience. Rather, they find out they have it after being tested for another health issue. Members of myPBCteam have shared how they went to the doctor for another issue before finding out about PBC.

“In the summer of 2023, I had Helicobacter. Once that was treated, I developed upper right quadrant pain,” explained one member. “I kept thinking it was my gallbladder, so I went to my doctor. It was my gallbladder but got a ‘two-for-one’ deal. The ultrasound shows my liver is normal, and I’ve started medication, and my numbers are coming down.”

Several genetic and environmental factors have been considered possible triggers for both conditions, but scientists have yet to determine the underlying causes.

3. Comorbidities and Complications

People with PSC are more likely to develop certain comorbidities (more than one health problem at the same time) or complications. For example, up to 80 percent of those with PSC also have IBD, especially ulcerative colitis (75 percent of the time). PSC is also linked to a higher risk of bile duct cancer, gallbladder cancer, liver cancer, and colorectal cancer (for those with IBD). However, these conditions aren’t always connected to PBC.

People with PSC and IBD need regular colonoscopy checks to catch colorectal cancer early. As found in the journal Pathologica, IBD alone increases colon cancer risk by tenfold as compared to the general population. Getting diagnosed with PSC in addition raises this risk again by fourfold.

Instead, people with PBC are most likely to have a variety of other autoimmune diseases. While PSC is linked to autoimmune diseases other than IBD about 25 percent of the time, 55 percent of people with PBC have another autoimmune disorder at the same time, such as thyroid disease or Sjögren’s syndrome. This higher rate isn’t that surprising when you consider that PBC is more common in females, who tend to get autoimmune disorders more often than males.

Some members of myPBCteam have discussed their history of autoimmune disorders. “I have had vague autoimmune symptoms for most of my life, including irritable bowels, dry mouth and eyes, skin rashes that come and go, loss of color along my knuckles, and fatigue,” said one member.

Others have shared memories of dry skin and itching that started in childhood.

4. Treatment Options for PSC and PBC

Currently, there are some treatments that cure PSC. There are some treatments for PBC, including ursodiol — also known as ursodeoxycholic acid or UDCA. UDCA modifies bile in a way that helps protect the cells and slow the disease progression.

Most people with PBC respond well to UDCA, but about 40 percent don’t have a strong enough response, so they require other forms of treatment like obeticholic acid (Ocaliva) or fenofibrate. With end-stage PBC and severe liver damage, a liver transplant can be a successful and lasting way to treat the disease.

Sometimes, people with PSC can also benefit from a liver transplant, but not if they’re very sick.

Liver transplants are not recommended for PSC if bile duct cancer has spread beyond the liver. Fortunately, new treatments are constantly being developed. Your doctor can help you manage the symptoms of PSC with medication to reduce itchiness, antibiotics for infections, and vitamins to treat nutrient deficiencies.

More Similarities Than Differences

Despite these differences, PBC and PSC have many characteristics in common. Not only do both conditions primarily affect the liver, but they’re also both rare diseases that can take time to diagnose and can significantly have an impact on your quality of life. It’s easy to feel alone when tackling a disease you’ve likely never heard of before.

On myPBCteam, members share honest feelings about their diagnosis. “At first, it was overwhelming and dooming,” explained one member. “Getting diagnosed was a relief that validated my symptoms, but there was not much support from family and friends. They couldn’t see a physical ailment and expected me to go on as they wished, even when I didn’t sleep or feel well. They really hesitated to talk about it or learn info about it. As time passed, I transformed into accepting my health issues.”

Learning that others can relate to your experience can help bring comfort and hope as you keep pushing ahead.

Talk With Others Who Understand

On myPBCteam, the social network for people with primary biliary cholangitis and their loved ones, members come together to ask questions, give advice, and share their stories with others who understand life with primary biliary cholangitis.

Have you been diagnosed with PBC? Do you have advice for others living with the condition? Share your experience in the comments below, or start a conversation by posting on your Activities page.