Your liver makes bile, a fluid that helps your body digest fats. If primary biliary cholangitis (PBC) damages the tiny tubes called ducts in your liver, bile can’t move through them. It can harden and form bile duct stones, which are painful and can get infected. Eventually, bile can back up inside your liver and cause scars.
You can take medicine to clear the bile out of your liver and reduce scarring. But if you don't treat PBC or it gets worse, you could have complications. These are the most common PBC complications and how to manage them.
Cirrhosis
Cirrhosis happens when scar tissue takes the place of healthy tissue in your liver. It's the late stage of PBC when your liver has a lot of damage. Cirrhosis is serious because it can lead to liver failure and liver cancer. PBC used to be called primary biliary cirrhosis because of this complication.
Your doctor might prescribe a medicine like ursodeoxycholic acid (Ursodiol), which removes bile from your liver and lessens liver damage. If you don't get much help from it, they may add obeticholic acid (Ocaliva) for your to take with ursodeoxycholic acid. Two new PPAR agonists, elafibranor (Iqirvo) and seladelpar (Livdelzi), are also now approved for use with ursodeoxycholic acid as well.
Fibrates are also an option, though they're prescribed off-label, meaning for a way other than originally approved by the FDA. Fibrates are a class of drugs that help lower lipids and have shown to be work well in patients who don't respond to ursodeoxycholic acid alone. They're often given with ursodeoxycholic.
If your liver has so much scar tissue inside that it doesn't work anymore, you may need a liver transplant. You'll get a healthy liver from a donor to replace your damaged one.
High Blood Pressure in the Liver Vein
The portal vein delivers blood to your liver. Scar tissue from cirrhosis can block this vein and cause blood to back up inside it. As more blood gets stuck inside the portal vein, pressure inside the vein rises. Doctors call this portal hypertension. It can cause bleeding inside your body if you don't treat it.
Medicines like beta-blockers lower pressure inside the portal vein. But a liver transplant is the only treatment that can stop portal hypertension.
Swollen Veins
When blood gets stuck in the portal vein, it can also back up into veins in your stomach and esophagus and make them swell. Doctors call these swollen veins varices. The pressure can build so much that the veins break open and release blood into the stomach and esophagus.
Bleeding varices are a medical emergency. You'll need treatment in a hospital. One way to stop the bleeding is to put an elastic band around the bleeding varices. You may get medicine through an IV to lower pressure in the portal vein.
Fluid Buildup in the Belly
Increased pressure in your portal vein can make fluid leak from your veins into your belly and collect there. Ascites is the name for fluid buildup in your belly.
One way to get rid of the extra fluid is to eat less salt and take water pills called diuretics. If these treatments don't help, your doctor may remove the fluid from your belly with a needle.
Weak Bones
PBC makes it harder for your body to build bone. Weak bones can easily break.
Your doctor can check your bone strength with a bone density test called a DEXA scan. They might also check the level of bone-building nutrients like calcium, vitamin D, and phosphorous in your blood.
Bisphosphonates and other medicines that treat osteoporosis may not work as well for people with PBC. Alendronate (Binosto, Fosamax) is the only bisphosphonate that seems to help.
To protect your bones, avoid alcohol and don't smoke. Try to do weight-bearing exercises every day. Take a walk, play tennis, or climb stairs. Get at least 1,200 milligrams of calcium and 800 international units of vitamin D daily from food or supplements. Estrogen therapy may help prevent bone loss in people with PBC who have gone through menopause.
High Cholesterol
The bile that your liver makes helps your body break down and absorb fats. Without enough bile, your body can't absorb fats very well. Extra fat could raise your low-density lipoprotein (LDL) cholesterol level. High LDL cholesterol is linked to an increased risk for heart disease.
Most people with PBC have high cholesterol. Your doctor might prescribe a statin drug or another medicine to lower your cholesterol if you also have other heart disease risks like these:
- Type 2 diabetes
- High body mass index (BMI)
- High blood pressure
- Smoking
Itchy Skin
Itching is a common problem in people with PBC. The itch can be intense enough to keep you awake at night and annoy you during the day. To stop the itch, here are some things to try:
- Shower in lukewarm water instead of hot water, and limit showers to 20 minutes or less.
- Gently pat your skin dry after you get out of the shower.
- Wear soft fabrics like cotton rather than itchy ones like wool.
- Use unscented detergents and gentle soap.
- Rub moisturizer on your skin every day.
- Keep your nails short so you won't injure your skin if you scratch.
Cholestyramine (Prevalite, Questran) is the only medicine that's approved to treat itching from PBC. Doctors sometimes prescribe other medicines that aren't approved for itching "off-label." These include:
- Bezafibrate: A drug that lowers cholesterol
- Gabapentin (Neurontin): Medicine that stops seizures
- Naltrexone (ReVia): Medicine that helps people stop drinking alcohol and using drugs
- Rifampicin (Rifadin): An antibiotic
- Sertraline (Zoloft): An antidepressant
Vitamin Deficiencies
When your body can't absorb fats well, it also can't absorb enough fat-soluble vitamins like A, D, E, and K. Too little of these vitamins can lead to deficiencies. If a blood test shows that you have low levels of these vitamins, you may need to take supplements to replace them.
Talking to Your Doctor
There's no cure for PBC, but treatments can slow the disease and prevent complications. You'll have regular checkups with your doctor to look for problems so you can treat them early. Between visits, tell your doctor if you have symptoms like these:
- Blood in your vomit or poop
- Confusion
- Itchy skin
- Swelling in your belly, lower legs, or feet
- Tiredness or weakness
- Weight loss without trying
- Yellow color in your skin or the whites of your eyes
Here are a few questions to ask your doctor:
- What symptoms might I have if my liver disease gets worse?
- What should I do if I have these symptoms?
- What tests will you do to find out if I have complications?
- How will you treat any complications that happen?
- What changes to my diet or lifestyle do you recommend?
Show Sources
Photo Credit: E+/Getty Images
SOURCES:
American Journal of Gastroenterology: "Changing Nomenclature for PBC: From 'Cirrhosis' to Cholangitis."
American Journal of Medicine: "Management of Pruritis in Primary Biliary Cholangitis: A Narrative Review."
Cleveland Clinic: "Ascites," "Esophageal Varices," "Portal Hypertension," "Primary Biliary Cholangitis."
Clinical and Experimental Gastroenterology: "Osteoporosis in Primary Biliary Cholangitis: Prevalence, Impact and Management Challenges."
Clinics in Liver Disease: "Evaluation and Management of Pruritis in Primary Biliary Cholangitis."
Columbia Surgery: "The Liver and Its Functions."
Frontline Gastroenterology: "Hyperlipidaemia in Primary Biliary Cholangitis: Treatment, Safety and Efficacy."
Johns Hopkins Medicine: "Portal Hypertension Treatment."
Journal of Clinical Densitometry: "The Pharmacologic Management of Osteoporosis in Primary Biliary Cholangitis: A Systematic Review and Meta-Analysis."
Mayo Clinic: "Primary Biliary Cholangitis."
NHS: "Treatment: Primary Biliary Cholangitis (Primary Biliary Cirrhosis)."
National Institute of Diabetes and Digestive and Kidney Diseases: "Symptoms & Causes of Cirrhosis," “Treatment of Primary Biliary Cholangitis (Primary Biliary Cirrhosis)."
PBCers Organization: "Questions to Ask Your Doctor."
StatPearls: "Primary Biliary Cholangitis."
World Journal of Gastroenterology: "Osteoporosis in Primary Biliary Cholangitis."
