Primary biliary cholangitis (PBC) is a rare liver disease in which the immune system attacks cells in the liver, causing damage that can threaten your health.
Fortunately, PBC can be treated successfully, allowing patients to live normal lives.
Hepatologist Colin Swales, MD, of Hartford Hospital, discusses the common questions his patients ask about PBC.
1. Q: How does PBC affect the body?
A: As a liver specialist, called a hepatologist, I treat patients with different liver conditions. The liver performs many different important functions in your body. It participates in the digestion of your food, helping to balance out your body’s energy needs. It also produces specialized proteins, carbohydrates, and other chemicals that help your body function normally. And it helps get rid of stuff your body doesn’t need by converting toxic substances into bile, a crucial fluid that helps you absorb nutrients from the food you eat.
With PBC, however, the immune system stops working correctly. Instead of doing its normal job and fighting off foreign substances in the body, your immune system actually attacks the parts of your liver that create bile, called your bile ducts. When your immune cells fight your bile ducts, they can get inflamed and severely damaged; without treatment, this damage can lead to permanent scarring in the liver, called cirrhosis. Cirrhosis increases your risk of liver cancer and other serious liver problems, which is why it’s so important to recognize and treat PBC as soon as possible.
2. Q: What are the symptoms of PBC?
A: Most people with PBC don’t experience any symptoms; physicians usually detect the condition because patients got a blood test done for another reason. However, in some cases, people suffer from intense itchiness all over the body, chronic fatigue, dry eyes and mouth, and other symptoms. We believe the itching occurs because the bile isn’t able to process substances the way it should, so irritating chemicals remain in the body and cause itching, also known as pruritus.
3. Q: How do you treat PBC?
A: I’ll often tell my patients PBC is a diagnosis I like to make, because we can do a lot about it. That’s not the case with all liver diseases, but this one is very treatable. Although patients will be treating their condition for the rest of their lives, by taking medicine, you’ll prevent liver damage and be able to go about your life as normal.
Medications for PBC calm inflammation, protecting against permanent damage and the need for a liver transplant. The most common medication for PBC is called ursodiol (Actigall) and it’s pretty well-tolerated by patients. Mild side effects range from hair, skin and nail changes to gastrointestinal symptoms like constipation or diarrhea. Serious side effects are very unlikely, and most patients do well by taking ursodiol as prescribed by their doctor.
However, between 15 and 30% of people don’t do well on ursodiol, and until very recently, there wasn’t much we could do for them. Fortunately, in 2016, a new medication called obeticholic acid (Ocaliva) was approved by the Food and Drug Administration (FDA) to treat PBC. Typically, we’ll prescribe obeticholic acid as an add-on treatment for patients while they also take ursodiol, but it can also be taken alone.
Obeticholic acid comes with some mild side effects, including nausea and diarrhea; additionally, patients can experience increased itchiness. There’s also a concern about cholesterol, because obeticholic acid tends to raise “bad” LDL cholesterol levels and lower “good” HDL cholesterol levels. Right now, studies are looking at this relationship to see whether it significantly increases heart disease risk, and we will know more in a few years. For now, as a hepatologist, I’ll weigh that concern against the potential downsides of liver disease and make that decision with my patients. It’s important that patients receive appropriate dosing of obeticholic acid, specifically if they have severe, end-stage liver disease, to prevent serious complications. In my practice, I’ve had good luck with obeticholic acid and I’m glad to have it as a backup option for patients who need it.
4. Q: What lifestyle changes can help keep patients with PBC healthy?
A: People with PBC often have vitamin deficiencies; bile helps our bodies absorb vitamins, and since PBC disrupts bile production, you may not be getting enough of vitamins A, D, E, and K. These vitamins play an especially crucial role in bone health, so people with PBC are at a high risk of osteoporosis, in which bones become weak and brittle. To prevent this, I recommend my PBC patients take vitamin supplements and increase their intake of calcium to keep bones strong. Osteoporosis can also be prevented by exercising, so patients should stay as active as possible. Additionally, smoking can damage your bones, so I encourage patients to quit.
Sometimes, treatments for PBC don’t relieve itchiness, so I’ll prescribe anti-itch medications and suggest that my patients try cool oatmeal baths and oatmeal creams.
It can be hard to learn you have a problem that could threaten your liver’s health in the long run, but I try to emphasize to patients that PBC can be successfully treated. Staying positive is important, as is maintaining a strong, trusting relationship with your hepatologist. Together, you and your doctor can control PBC so you can live a normal life.
