Primary biliary cholangitis (PBC) is a type of autoimmune disease that happens when the immune system, which usually fights off infections, starts attacking healthy parts of the body instead. In PBC, the target is the liver’s small bile ducts (tiny tubes). These ducts are important because they transport bile, a digestive fluid that also helps eliminate toxins, into the intestines. This ongoing attack can lead to serious liver damage over time. Eventually, this might turn into cirrhosis, where the liver is permanently scarred and doesn’t work properly. If you have PBC, you might also be curious about whether it’s related to other autoimmune diseases, like lupus.

Studies from the Lupus Foundation of America have found that having lupus or PBC increases a person’s chances of developing the other condition. However, it’s still uncommon for people to have both PBC and lupus at the same time.

This article explores the relationship between lupus and PBC, including key facts about lupus, whether lupus can cause cirrhosis, and how to manage lupus if you have PBC.

What Is Lupus?

Lupus is another autoimmune disease that can affect various parts of the body, including the skin, joints, kidneys, and even the brain. The most common type, systemic lupus erythematosus (SLE), affects multiple organs. Other forms include cutaneous lupus, which mostly affects the skin, and drug-induced lupus, which is triggered by certain medications.

Causes of Lupus

The cause of lupus is unknown. Most studies suggest genetics, hormones, and the environment play a role. Specifically, having certain genes may make you more likely to develop lupus. Your body’s reaction to certain hormones, such as estrogen, could also increase your risk of lupus. Environmental triggers like exposure to ultraviolet light and infections (especially Epstein-Barr virus) may also affect your chances of getting lupus.

Anyone can develop lupus, but certain groups of people are more at risk. According to the Lupus Foundation of America, these include:

• Women who are 15 to 44 years old
• People who are African American, Asian American, Hispanic/Latino, Native American, or Pacific Islander
• People with a family history of lupus or another autoimmune condition

Recognizing Lupus Signs

Lupus symptoms can vary greatly depending on the person, the type of lupus, and the body part affected. Some of the more common symptoms include:

• Feeling tired all the time (fatigue)
• Painful or swollen joints
• Skin rashes (especially a butterfly-shaped rash on the face)
• Fever with no known cause
• Headaches
• Swelling in the feet, hands, or around the eyes
• Chest pain when taking a deep breath
• Sensitivity to sunlight

When you have lupus, symptoms can come and go. When they’re bothering you, it’s called a flare. Flares can be mild or severe, and new symptoms can occur at any time.

Lupus Diagnosis and Treatment

Diagnosing lupus can be difficult, as its symptoms are like many other health conditions and can appear and disappear. There’s also no specific test that can tell if you have lupus. Health care providers typically use a combination of medical history, physical exams, blood tests, and biopsies (removing a small piece of tissue for examination) to diagnose it.

Lupus has no cure, but there are many treatment options to relieve symptoms, prevent flares, and decrease organ damage. These may include medications such as steroids, nonsteroidal anti-inflammatory drugs (NSAIDs), immunosuppressants, and antimalarial drugs.

If you have lupus, you may need to see multiple health care providers. Your primary care provider and a rheumatologist (a doctor specializing in joint and muscle diseases) are typically involved. Depending on how lupus affects your body, you may also need to see other specialists. For example, you may need a cardiologist if lupus damages your heart or blood vessels. The type of specialist you see will depend on your needs.

Impact of Lupus on Daily Life

Living with lupus can be challenging, as symptoms can change and aren’t easy to describe. Fatigue and pain can make it difficult to perform daily tasks, while the unpredictability of flare-ups may require changes to your social, family, and work life. That’s why finding ways to cope with these changes and the stress they may bring is vital. Coping strategies that may help include calming activities, regular physical activity, or building a solid support system.

Exploring the Link Between Lupus and PBC

There hasn’t been much research on cirrhosis in people with lupus. Cirrhosis is a stage of chronic liver disease where there is permanent scarring of the liver. Current studies show that 19.4 percent to 60 percent of people with SLE may have liver problems at some point in their lives. However, among those with SLE who do have liver disease, only about 1 percent to 2 percent end up with cirrhosis.

PBC has some similarities with lupus, which suggests that it might be an autoimmune disease as well. However, although PBC is sometimes found with other autoimmune disorders, it’s rare for people to have both PBC and SLE. Studies show that only a small percentage of people with PBC — roughly 0.5 percent to 3.7 percent — also have SLE.

What Causes Lupus in People With PBC?

A 2024 study from the journal Arthritis Research & Study looked at people living with SLE and PBC to determine whether there’s a potential cause-and-effect relationship between the two conditions. Researchers found that having one condition increases the risk of getting the other.

They also found that people with SLE and PBC share some common genes. Specifically, they found nine genes that could help us understand more about how SLE and PBC are related and help identify new treatments for people with both conditions.

However, we need more research to better understand how the two conditions interact and to find new treatments for those with SLE and PBC.

Treating Lupus With PBC

Because lupus and PBC rarely occur at the same time, researchers don’t fully understand how to treat both conditions together or if treatments for one condition can affect the other.

However, lupus or the medications used to treat it can cause severe liver problems like hepatitis (inflammation of the liver) and vasculitis (inflammation of blood vessels in the liver). These issues can make the liver damage from PBC even worse. That’s why people with both conditions need to work closely with their health care team to figure out the best treatment plan. Your health care team will include your primary care physician as well as specialists for liver, gut, and autoimmune diseases. Your provider might need to balance medications for lupus and PBC to avoid making liver problems worse while treating lupus symptoms.

Despite these challenges, various treatment options are available for lupus as well as PBC. However, more research is needed to determine whether these treatments may interfere with each other or increase the risk of complications in people with PBC.

If you have lupus and PBC, stay in touch with your health care team and ask them about your symptoms, concerns, and treatment options. Working together, you and your doctor can develop a plan to manage both conditions effectively. By keeping track of any changes in how you feel, you can stay as healthy as possible and feel your best.

Find Your Team

On myPBCteam, the social network for people and their loved ones living with primary biliary cholangitis, 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 lupus and PBC? Do you have any questions? Share your experience in the comments below, or start a conversation by posting on your Activities page.