If you’ve been navigating life with primary biliary cholangitis (PBC), formerly known as primary biliary cirrhosis, you know it can be tough to manage your symptoms. One issue you might face with PBC is high cholesterol.

At myPBCteam, we’re here to help. We’ll explain how PBC and cholesterol are connected and what you can do about it.

What Is High Cholesterol?

Cholesterol is a fat (lipid) carried through your blood via proteins. Together, cholesterol and its proteins are called lipoproteins. You need small amounts of cholesterol to help build cells and make hormones and vitamin D.

If there’s too much cholesterol, it can build up as plaque in your blood vessels (atherosclerosis). Over time, enough plaque buildup can block your blood vessels. Blocked arteries put you at risk of a heart attack and stroke. These changes are called cardiovascular disease.

You may have heard of good cholesterol and bad cholesterol. Low-density lipoprotein (LDL) is the bad cholesterol because it can build up in your blood vessels. High-density lipoprotein (HDL) is the good cholesterol because it brings extra cholesterol to the liver to remove it from your body. People with PBC also have an abnormal type of cholesterol called lipoprotein X.

You’re probably wondering what this has to do with PBC. Keep reading to find out how the two are related.

How Are High Cholesterol and PBC Connected?

People with PBC are at an increased risk of having too much cholesterol. High cholesterol — also called hypercholesterolemia or hyperlipidemia — affects 75 percent to 95 percent of people with PBC. In comparison, only about 10 percent of adults in the U.S. have high cholesterol.

How high cholesterol develops in people with PBC is complicated. On one hand, as PBC progresses, your bile ducts may block bile flow through your liver (cholestasis). Bile helps break down and absorb dietary fats, including cholesterol from the small intestine. So, people with PBC can’t break down all the cholesterol.

On the other hand, your liver makes cholesterol in response to how much cholesterol is absorbed. Think of your liver like a doting grandmother. She always vacuums up the crumbs after you eat cookies. But if there are no crumbs, Grandma might think that she didn’t make you enough cookies and rush to put another batch in the oven. The liver works the same way, but instead of cookies, it cleans up and makes cholesterol.

In PBC, less cholesterol is broken down and absorbed. So, the liver sees no crumbs and thinks you don’t have enough cholesterol. The liver starts making even more cholesterol to compensate in a process called intrahepatic cholesterol synthesis.

If you have PBC, less cholesterol is removed and more cholesterol is made. That’s a recipe for too much cholesterol and a higher risk of cardiovascular disease.

How Do Doctors Test for High Cholesterol?

You might be wondering if you can tell whether you have high cholesterol based on how you feel. However, there are no symptoms of high cholesterol. You’ll most likely find out you have high cholesterol through a standard blood test. Most people are screened for cholesterol every few years, regardless of whether they have PBC.

If you have PBC, your doctor will want to do a special type of cholesterol blood test called ApoB-100. This test is more specific for people with PBC than the standard cholesterol tests because it accounts for lipoprotein X. If your ApoB-100 concentrations are low and you have no cardiovascular risk factors, like diabetes, high blood pressure (hypertension), or tobacco use, the doctor will follow up every five years or so.

Your doctors will be more careful if you have other risk factors for cardiovascular disease. Lower ApoB-100 numbers will worry them. If you have good cholesterol levels but risk factors, they’ll want to recheck you every year. If your doctors find your cholesterol is high, they’ll probably want to start treatment to lower those levels right away.

Sometimes, high cholesterol is only discovered after someone already has heart disease. One myPBCteam member reported, “Coronary artery disease was discovered by accident after a scan.”

The good news is that high cholesterol is treatable.

How Can High Cholesterol Be Managed?

Your doctor will most likely start you on a moderate-intensity statin like atorvastatin or simvastatin. Statins work by blocking a material that makes cholesterol. You’ll take your statin as a pill every day. After a few months, your doctor will check your ApoB-100 levels. If the cholesterol levels haven’t gone down enough, they may increase your dose.

If after a few more months, there still isn’t an improvement in your cholesterol levels, you may be prescribed an additional drug. PCSK9 inhibitors are a new type of cholesterol drug that helps your liver clear the bad cholesterol from your blood. You’ll likely get an injection of PCSK9 inhibitor at your doctor’s office every few months.

The PBC treatment you’re already taking may also help lower your cholesterol. Ursodeoxycholic acid (UDCA or Ursodiol) is a bile acid that helps your liver move bile. UDCA treats cholestasis, the underlying cause of high cholesterol, so your total cholesterol levels go down.

Fenofibrate is another PBC treatment that helps lower cholesterol. However, researchers aren’t sure that fibrates protect you against heart disease.

A healthy lifestyle is a key part of managing your risk of cardiovascular disease. Make sure you eat healthy foods, exercise regularly, and get plenty of sleep. These healthy habits should go along with the cholesterol-lowering medications you take.

On myPBCteam, members discuss what treatments work for them. One member wrote, “I myself take a statin. A lot of people with PBC are also dealing with high cholesterol as part of it, and it’s important to treat that so you don’t end up with heart problems.”

Are Cholesterol-Lowering Medications Safe for People With PBC?

Many medications are processed in your liver. Researchers have worried that statins and other medications may be toxic for people with PBC whose livers are already damaged. Although research is still limited, studies so far have shown that statins are safe in people with PBC. PCSK9 inhibitors seem to be safe in people with moderate liver disease. Your doctors will help you understand your risk and closely track your hepatic (liver) health.

One myPBCteam member points out the importance of collaboration between your doctors: “Are your general practitioner and your hepatologist talking together? I would definitely check any new medication or supplement with your hepatologist. I just feel that they’re the ones that would know the answer the best.”

What Are the Side Effects of Cholesterol Medications?

Common side effects of statins are headache, dizziness, tiredness, and digestion problems. But every person tolerates statins differently. Talk to your doctor if your side effects affect your daily life. They may change your dose or switch you to a PCSK9 inhibitor.

If you’re taking a PCSK9 inhibitor, you may feel fatigue and muscle soreness. As with most shots, the site of your injection might be sore or swollen, too. UDCA has few side effects, but you may gain weight, have diarrhea, or lose hair.

Preventing and treating high cholesterol is an important consideration for people with PBC. Your doctor can help you understand why you have high cholesterol and find the best way to manage it. It might take some trial and error, but once you find a strategy that works for you, your overall health will improve.

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.

Have you been diagnosed with PBC? Do you have high cholesterol? Share your experiences in the comments below, or start a conversation by posting on your Activities page.