Primary Biliary Cholangitis (PBC)

Symptoms of PBC can develop slowly, and many patients have no symptoms at first. The initial symptoms of PBC are:

• Itchy skin
• Fatigue
• Belly pain
• Dry mouth and eyes

As the disease progresses, complications of cirrhosis and portal hypertension (high pressure within the blood vessels that drain into the liver) may develop. In addition, osteoporosis (thinning of the bones), fat soluble vitamin deficiency (vitamin A, D, E, K) and fat deposit-forming nodules on the eyelids (xanthelasma) or at the joints and buttocks (xanthoma) are common complications of PBC.

It’s believed that an overactive immune system causes primary biliary cholangitis, but what triggers this over-activity still isn’t clearly known. A genetic predisposition for PBC likely exists, and exposure to certain bacteria, viruses or toxins is believed to somehow trigger the immune system, which reacts in an inappropriate way and causes injury to the bile ducts. PBC affects mostly women.

Your doctor will do a complete health history and physical exam. In order to accurately diagnose primary biliary cholangitis, your doctor may use the following methods:

• Blood tests to see whether you have abnormal liver enzymes (an increase in these enzymes is due to damaged bile ducts). Your doctor will also test you for anti-mitochondrial antibodies (AMAs), which are almost always present in patients with PBC – so the diagnosis can usually be made based on blood tests alone
• Elastography
  • Transient Elastography (Fibroscan®) is a special ultrasound that measures the amount of fat in the liver and the stiffness of the liver, which are indirect measures of the amount of scarring present.
  • MR Elastography is a special way of obtaining an MRI that also measures the amount of fat in, and stiffness of, the liver.
• Liver biopsy. A small sample of liver tissue is taken and checked under a microscope. It can confirm the diagnosis of PBC and provide information on the presence and severity of scar tissue. It also allows your doctor to examine your liver for other diseases.

Although primary biliary cholangitis can’t be cured, its progression can be slowed with ursodeoxycholic acid (UCDA) and obeticholic acid, medications that can help improve the bile flow in your liver. Your health care provider can also treat your PBC symptoms; cholestyramine, colestipol hydrochloride, antihistamines, rifampin and naltrexone are all medicines that may be used to treat the itching caused by PBC.

Vitamin supplements can correct deficiencies in fat soluble vitamins that you may have. Osteoporosis is common in patients with primary biliary cholangitis, and your bone density will need to be monitored by your doctor. Cholesterol problems also tend to develop in patients with advanced stages of the disease; you may need treatment to avoid additional complications such as heart disease or stroke.

If primary biliary cholangitis eventually causes your liver to fail, your doctor may recommend a liver transplant. Liver transplants can cure primary biliary cholangitis, although PBC may recur in the new liver in a small number of patients.