By Evelyn Creekmore
Primary biliary cholangitis (PBC), also known as primary biliary cirrhosis, is a chronic, autoimmune disorder that damages the liver’s bile ducts. PBC causes bile to build up instead of moving on through the ducts to the gall bladder and small intestine as it should to help digestion. Up to 60% of those with PBC are diagnosed before they have any symptoms, and they may not develop symptoms for years. That doesn’t mean you don’t need PBC treatment. It’s important to know that PBC is progressive. Whether or not you have symptoms, PBC progressively advances to more serious stages, causing complications that involve your entire body, unless treatment slows the progression.
Osteoporosis is a thinning of the bones that can cause them to break easily. While the exact relationship between osteoporosis and PBC is not yet known, osteoporosis is the most common complication of PBC. To support your bone health, your doctor may recommend an initial bone density scan or prescribe calcium and vitamin D supplements.
2. Vitamin Deficiencies
Because bile is blocked from flowing to the small intestine, it can’t help digest the fat-soluble vitamins your body needs. Your doctor can do a simple blood test to check for vitamin A, D, E, and K deficiencies and prescribe supplements as needed.
As bile builds up, it can harden into painful gallstones that may lead to infection. Specific reasons your doctor may prescribe medication are to break up these stones or to help prevent stones from forming.
4. Fat Malabsorption
PBC often causes high cholesterol that can cause yellow, bumpy fat deposits under your skin, typically around joints and eyes. Fat malabsorption usually occurs when PBC is very advanced. Symptoms include diarrhea, oily stool, and unexplained weight loss. Your doctor can recommend specific lifestyle changes and medication to help keep your cholesterol in check.
Jaundice causes your skin and the whites of your eyes to yellow. Like fat malabsorption, jaundice usually doesn’t occur until PBC is very advanced and the liver has stopped working normally.
6. Cirrhosis of the Liver
Cirrhosis only occurs in the most advanced stage of PBC, or stage 4, when scarring has spread to the whole liver, and there is a danger of liver failure. Cirrhosis represents a turning point in the disease toward its most serious complications.
7. Portal Hypertension
Scarring from cirrhosis prevents the portal vein from delivering blood to your liver from other vital organs, a complication called portal hypertension. Your doctor may prescribe a beta blocker to help lower your blood pressure or a nitrate to help improve blood flow.
Once blood builds up in the portal vein, it can back up all the way to your stomach or even esophagus, a life-threatening condition called varices (enlarged veins). Ask your doctor about regular screening for varices and seek immediate medical attention if they develop. Signs of varices include vomiting blood or passing bloody or black stools.
9. Liver Cancer
Having cirrhosis increases the risk of liver cancer. For those with PBC, liver cancer screening is recommended every 6 to 12 months. Many effective treatments for liver cancer are available, especially if treatment begins before the cancer spreads.
10. Liver Failure
Liver failure caused by PBC can only be reversed with a liver transplant. In the United States, between 5,000 and 6,000 liver transplants are performed every year. Most people feel “back to normal” within six months to a year of a successful transplant. Liver transplant greatly improves survival in patients with advanced PBC. Approximately 30% of liver transplant recipients may develop recurrent PBC although aggressive use of immunosuppressive drugs following transplant can lower the risk of PBC recurrence.
Although the complications of PBC are well understood in the medical community, the cause of the disease is not. Research points to a genetic component. You may have done nothing to get the disease, but there is much you can do to lessen its impact on your life. Start by talking with your doctor about the screening and treatment options that are right for you.