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Have you ever felt like your diagnosis didn’t tell the whole story? Maybe your symptoms changed, or your treatments didn’t work as well as you’d hoped. If you’re living with primary biliary cholangitis (PBC), you may have heard of another liver condition called autoimmune hepatitis (AIH). Though rare, it’s possible for people with PBC to develop features of this second liver disease.
PBC and AIH are both types of autoimmune liver diseases, but they affect your liver in different ways. In this article, we’ll break down what autoimmune hepatitis is, how it’s connected to PBC, and what to know if you have both. We’ll also walk you through some treatment options, symptoms to watch for, and ways to manage both conditions.
Autoimmune hepatitis is a type of chronic liver disease. It develops when your immune system, which normally works to fight germs and viruses, attacks your liver cells by mistake. This causes inflammation in the liver, which can lead to fibrosis (liver damage that leads to scarring) and cirrhosis (advanced scarring) if not properly treated.
Over time, AIH can affect how well your liver works. When the liver can’t do its job, it may struggle to filter toxins, make proteins, or produce bile. This can lead to serious problems like:
The answer to this question is no. Unlike some other types of hepatitis, AIH isn’t caused by a virus or something you can catch from someone else. It’s a long-term condition that can be triggered by genes, lifestyle factors, and other situations. Unfortunately, there’s no cure yet. However, with proper and timely treatment, many people go into remission — periods when symptoms are controlled — and live healthy lives.
It’s not always clear why someone gets AIH. However, doctors believe it happens when something triggers the immune system to attack the liver. This could be an infection, certain medications, or even something in the environment. In people with a genetic risk, certain triggers may confuse the immune system into thinking liver cells are harmful. Instead of protecting the body, the immune system mistakenly causes inflammation and liver damage.
Some people with AIH have no symptoms at all and only find out after abnormal results from routine blood tests. For others, symptoms may begin slowly or appear suddenly. Early symptoms include:
As the disease progresses, new symptoms may appear as a result of worsening liver function and bile building up in the bloodstream. These can include:
There’s no single test that can confirm a diagnosis of AIH. Instead, your doctor may use a combination of blood tests, imaging tests, and possibly take a liver biopsy (removing a sample of liver tissue to be examined under a microscope).
Blood tests help check levels of liver enzymes, which are usually much higher in people with AIH. Other tests check for autoantibodies — immune system proteins that attack the body’s own healthy cells. These proteins include antinuclear antibodies (ANA) and anti-smooth muscle antibodies (ASMA).
Other blood tests may be used to rule out similar diseases, such as viral hepatitis or Wilson disease. Imaging tests and scans can also be used to reveal signs of cirrhosis or bile duct problems like primary sclerosing cholangitis. If a biopsy is taken, tissue can then be examined under a microscope to check for inflammation, scarring, or other features unique to AIH.
In PBC, the small bile ducts of the liver — tiny tubes that carry bile to the small intestine — are affected. AIH, on the other hand, targets the liver cells themselves. In rare cases, a person can have signs of both conditions. This is called overlap syndrome.
Overlap syndrome is uncommon, and most of what we know comes from case reports (studies based on a single individual) or other small studies. Researchers estimate that about 2 percent to 19 percent of people with PBC may also show features of AIH.
Having PBC doesn’t mean you’ll definitely get AIH. However, since both are autoimmune disorders, having one can increase the chances of getting the other. In general, people with an autoimmune disease have about a 25 percent to 50 percent chance of eventually developing a second autoimmune condition.
To diagnose overlap syndrome, doctors look for features of both conditions. According to accepted guidelines, a person must meet at least two out of three criteria for each disease, and diseases that have similar presentation need to be excluded, like viral hepatitis and alcoholic liver disease.
For PBC, the diagnostic criteria are:
To diagnose AIH, the criteria are:
People with overlap syndrome often show a mix of symptoms and lab results from both diseases. For example, they might have fatigue and itching, like in PBC, but also have high ALT, AST, or IgG levels more typical in AIH.
While overlap syndrome is rare, if you’ve been diagnosed with PBC, it’s important to watch for signs of AIH. This is because people with both conditions may have a faster progression to cirrhosis, liver failure, or complications like ascites compared to people with PBC alone. Catching AIH early allows for faster treatment and may help prevent serious liver damage.
AIH and PBC can sometimes appear at the same time. More often, however, AIH develops after PBC. The second diagnosis can happen months or even years later. This means that ongoing monitoring is important, even if your PBC has been stable.
Warning signs may include a sudden jump in ALT or AST levels — liver enzymes more closely associated with AIH. Your doctor may also use antibody tests to check for high IgG, ANA, or ASMA levels. If needed, a liver biopsy can confirm the diagnosis of overlap syndrome.
Treating PBC usually begins with Ursodiol, also known as ursodeoxycholic acid or UDCA. This medication helps improve bile flow, reduce liver enzyme levels, and slow down fibrosis. People who don’t respond well to or can’t tolerate UDCA because of side effects may be prescribed obeticholic acid (Ocaliva), elafibranor (Iqirvo), or seladelpar (Livdelzi) next. Treatment with one of these medications is long term and can help delay serious complications, such as cirrhosis or the need for a liver transplant.
Autoimmune hepatitis treatments are different. Since the condition involves the immune system attacking liver cells, treatment usually includes immunosuppressive medications. These might include corticosteroids — such as prednisone or budesonide — or the immunosuppressant azathioprine. These medicines can’t offer a complete cure, but they aim to bring the disease into remission and prevent further liver damage.
When a person has overlap syndrome, treatment often combines both approaches. UDCA is still used to support bile flow, while steroids or immunosuppressants may be added to calm the immune system and reduce inflammation. The specific treatment plan depends on how severe the AIH features are and whether liver enzymes like ALT or AST are too high.
Managing both conditions often requires lifelong monitoring. Your doctor will regularly check your liver enzymes, antibody levels, and symptoms to adjust treatment as needed.
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 tips with others who understand life with primary biliary cholangitis.
Are you living with primary biliary cholangitis and autoimmune hepatitis? What tests led to your diagnoses, and what treatments have worked best for you? Share your experiences in the comments below, or start a conversation by posting on your Activities page.
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