Living with primary biliary cholangitis (PBC, previously known as primary biliary cirrhosis) often means dealing with flare-ups. Even if you find an effective treatment, PBC symptoms can flare occasionally. It’s easy to feel frustrated or disconcerted when that happens, but you can take several steps to help yourself feel better.

Talking to a health care provider about your PBC symptoms is one of the best ways to better prevent and manage them. Here’s what you need to know about PBC flare-up symptoms and how to manage them when they occur.

What Is a Flare-Up?

In PBC, as with many other autoimmune diseases, symptoms may come and go. Symptoms may be around for a while and then leave, and they may not come back for some time (called remission). Periods when symptoms do return or get worse are called flare-ups or flares. Although research is still in the early stages, it seems that many autoimmune diseases flare up when specific immune system mechanisms get activated or triggered.

Triggers of autoimmune condition flare-ups include:

• Stress
• Exposure to certain chemicals
• Infections
• Other (sometimes unknown) environmental factors

What Does It Mean When Primary Biliary Cholangitis Flares Up?

When PBC flares up, your symptoms will get worse for a while. It’s important to be aware of all the common symptoms of PBC. That way, you have a good chance of recognizing a flare-up early even if you experience different symptoms than before.

What Causes a PBC Flare-Up?

Researchers aren’t entirely sure what causes PBC or brings on flares. They believe a combination of environmental and genetic factors influences the development of PBC.

Researchers have linked PBC with immune system changes, which may influence flares. People with PBC have lower numbers of immune cells called T cells. Their T cells also work differently from those of people without PBC. It’s possible that these changes influence PBC symptoms, but more research is needed to understand the connection between T cells and PBC symptoms.

With PBC, the immune system mistakenly attacks the body’s own tissues rather than foreign invaders like bacteria or viruses. These mechanisms may also be related to PBC symptom flares, but the evidence isn’t clear.

PBC Flare-Up Symptoms To Pay Attention To

While the causes of primary biliary cholangitis and flares aren’t clear, researchers have a better understanding of its symptoms. Here are some of the most common PBC symptoms that you may experience during a flare-up.

1. Itchiness

Itchy skin (medically known as pruritus) is common in people with PBC. A 2023 study indicated that 81 percent of 170 participants with PBC experienced itching at some point, although a 2018 study of more than 1,600 people with PBC put that number at 73.5 percent.

On myPBCteam, several people have reported itching when they have a flare-up. They’ve also noted that their itching gets better when the flare goes away. One member said, “Woke up not feeling as itchy today after an almost two-week flare-up. That’s a slight win!”

2. Fatigue

Fatigue is another common symptom of PBC. A 2022 study reported that 50 percent of people living with PBC experience fatigue, and 20 percent have severe fatigue that negatively affects their quality of life.

“The fatigue is a struggle,” said one member of myPBCteam.

Fatigue is such a common experience that another shared, “Had a good day, just the usual fatigue.”

3. Jaundice

If you have severe PBC, you may experience jaundice — yellowing of skin and the white part of the eye that happens when liver damage is so severe that this organ can’t function properly. Jaundice occurs in at least 25 percent of people with PBC, according to the World Journal of Gastroenterology. One PBCteam member described their flare: “[My] eyes were yellow, and I had nausea for a week.”

4. Dry Eyes or Dry Mouth

Somewhere between 47 percent and 73 percent of people with PBC experience dry eyes or dry mouth. These symptoms are usually caused by Sjögren’s syndrome, which is common in people with PBC. Study findings describe a wide range of prevalence (commonness) — from 3.5 percent to 73 percent — of Sjogren’s in people who have PBC.

The conditions can flare at the same time, so symptoms of Sjögren’s may happen alongside symptoms of PBC.

5. Pain or Swelling

A PBC flare may also start with pain and swelling in the abdomen or elsewhere. PBC can cause pain in the liver and the spleen, which are both in the abdomen. Gallbladder problems like gallstones and stones in the bile ducts may also occur and can be painful.

You may experience swelling and pain elsewhere. Your bones, joints, or muscles may hurt, and you may develop edema (swelling in the feet and ankles). Whether pain and swelling are related depends on their cause, but these symptoms often occur together. As one myPBCteam member said, “I am having some kind of flare-up with swollen and sore knees and heavy legs.”

How To Manage Flare-Ups of Primary Biliary Cholangitis

You can take several approaches to help manage PBC symptoms when they flare.

1. Avoid Certain Foods and Drinks

Making some changes to your diet may help you feel better when you’re having symptoms of a PBC flare. Avoiding added sodium (salt), raw shellfish, and alcohol may help prevent worsening symptoms and other PBC–related problems.

Members of myPBCteam have discussed dietary habits to help manage symptoms. “I’m eating pickled things, but I have to be careful of salt,” one said.

Another mentioned, “I don’t drink alcohol. I was never a big drinker, but I do miss it on occasion.”

2. Don’t Use Cigarettes

According to Mayo Clinic, environmental factors like smoking cigarettes are potential triggers for PBC. Your doctor may recommend that you not smoke if you have PBC. If you currently smoke and want to quit, talk to a health care provider about your options.

3. Treat Infections Quickly

If you think you have an infection of any sort, talk to your health care provider right away. They’ll assess your symptoms and suggest treatment. Infections can be a hazard for people living with a liver disease like PBC, so treating them quickly will help you feel more equipped to manage PBC symptoms.

4. Practice Self-Care

Taking good care of yourself can help you feel better and keep symptoms of PBC at bay. You self-care plan may include:

• Learning more about PBC
• Finding people you trust and asking them for help
• Spending time with friends and family
• Getting plenty of rest
• Exercising when you can
• Eating well
• Taking steps to help reduce stress

Choose techniques that feel good for you and fit into your lifestyle. Regular self-care can help you maintain a high quality of life and sense of well-being, so you’re best prepared to deal with PBC flares when they happen.

5. Talk to Your Health Care Provider

If you are concerned about a PBC flare, experience regular flares, or think you might be having one, talk to your doctor. They should be able to help you understand your flares and what might be causing them. In addition, they can explain all your PBC treatment options, including medications — like steroids (budesonide), ursodiol (ursodeoxycholic acid, or UDCA), and obeticholic acid (Ocaliva) — liver transplant, and more.

Read more about specific medications and procedures in this list of treatments for PBC.

Health care providers can perform blood tests or do a liver biopsy to examine your liver tissue and determine what’s going on with your PBC. With more information about your condition, they can help you understand your treatment options and best next steps. Routine testing will also help your doctor track the progression of your PBC and prevent complications like liver failure.

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 tips with others who understand life with primary biliary cholangitis.

Are you wondering if the symptoms you’re experiencing might be a PBC flare-up? What are some ways you manage symptoms of a flare? Share your experiences in the comments below, or start a conversation by posting on your Activities page.