Primary Biliary Cholangitis(PBC)
Come See the Experts in the Care of PBC
Primary biliary cholangitis (PBC) is a rare, chronic, progressive inflammatory disease that slowly destroys the small bile ducts in the liver and may eventually cause the liver to stop functioning properly. At NewYork-Presbyterian, our liver specialists understand the complexity of PBC and its potential complications and offer customized treatment to relieve symptoms and slow their progression. As a leading center for liver transplantation, we have exceptional experience offering this treatment to people with advanced PBC.
What causes primary biliary cholangitis?
It is not completely known was causes PBC, but it is far more common in women than men and is thought to be an autoimmune disease. The immune system may attack healthy cells in the bile ducts. A combination of genetic and environmental factors may trigger this autoimmune response.
PBC Signs & Symptoms
Because primary biliary cholangitis develops slowly, most patients do not have any symptoms when they are diagnosed. But it can be detected by abnormal liver blood tests and then confirmed often without any need for invasive testing. It can take 5-20 years to develop symptoms, which may include:
- Darkening or discoloration of the skin
- Dry eyes and mouth
- Itchy skin
Later symptoms may include the following:
- Abdominal pain
- Fatty deposits around the eyes, eyelids, or in the creases of the palms, soles, elbows, or knees (xanthomas)
- Abdominal swelling
- High cholesterol
- Jaundice (yellowing of whites of the eyes and the skin)
- Memory problems
- Swollen feet and ankles
- Weight loss
Primary Biliary Cholangitis Diagnosis
Your doctors perform a full assessment to determine if you have primary biliary cholangitis, including:
- Physical examination
- Blood tests
- Fibroscan® (a special ultrasound that helps determine the amount of scarring in the liver)
- Liver biopsy (examination of a small amount of liver tissue) is required to rule out other causes of liver disease
- Magnetic resonance imaging with cholangiography (MRCP) and elastography (MRE) to assess scarring
- CT scanning
Our Approach to Care
Your healthcare team includes specialists who diagnose and treat PBC, including hepatologists (liver doctors), gastroenterologists, interventional endoscopists, liver surgeons, physician assistants, nurses, registered dietitians, social workers, and others. All of the healthcare professionals you may need are available to you through one medical center.
Treatment of primary biliary cholangitis
While there is no cure for primary biliary cholangitis, NewYork-Presbyterian provides treatments designed to slow the progression of your disease, relieve your symptoms, and prevent or treat complications so you can live a longer, more comfortable life.
Medications. Doctors may prescribe the drug ursodeoxycholic acid (UDCA), a naturally occurring bile acid, to increase the flow of bile and reduce inflammation of the bile ducts. If you do not respond to UDCA, your doctor may add another medication called obeticholic acid. Recent research has also shown that fibrate medications (such as fenofibrate) can help.
Nutritional supplements. People with PBC are at risk for nutritional deficiencies due to poor absorption of nutrients. You may receive vitamin and mineral supplements to replace missing nutrients, guided by our expert dietitians.
Evaluation for osteoporosis. Your doctors will monitor your bone density because people with PBC are also at risk for osteoporosis. We utilize the latest medical therapies to treat and prevent this complication.
Liver transplantation. If your PBC becomes so advanced that it cannot be effectively managed with other treatments, you may need a liver transplant. At NewYork-Presbyterian, we utilize a variety of liver transplant approaches, including living donor liver transplantation.
Treatment of PBC complications. Portal hypertension (increased pressure in the portal vein, the major vein transporting blood from your stomach to your liver and other digestive organs) is a serious complication of PBC. Some people develop varices (enlarged veins in the stomach or esophagus). At NewYork-Presbyterian, your team includes specially trained doctors who can treat these complications.
Why Choose Us
Primary biliary cholangitis is not a common disease, and doctors at some other hospitals may not be as familiar with its treatment. At NewYork-Presbyterian, you'll have access to a team with expertise in this condition that offers all of the services you need. Because people with PBC may also have certain metabolic and autoimmune disorders — such as thyroid disease, rheumatoid arthritis, CREST syndrome (an immune disorder that is a type of scleroderma, a thickening of connective tissue), and Raynaud's disease (a condition that affects small blood vessels in the hands and feet in response to cold or emotional stress) — your team may also include doctors to treat those conditions, too. Contact us to schedule an appointment.