Primary biliary cirrhosis (PBC) is a rare, chronic, progressive inflammatory disease which slowly destroys the small bile ducts within the liver, and which eventually can cause the liver to stop functioning normally.
The slow inflammatory destruction of the liver's bile ducts causes toxins and bile to build up in the liver, and leads to irreversible liver scarring, called cirrhosis, liver failure, and the need for a liver transplant. Women have a much higher incidence of PBC than men: 90% of PBC patients are women.
NewYork-Presbyterian's digestive disease team includes a number of specialists with extensive experience treating PBC who can help slow the progression of the disease and preserve as much liver function as possible. We are also home to a world-renowned Center for Liver Disease and Transplantation, which enables us to treat end-stage PBC with liver transplantation.
While the cause of PBC is not certain, most experts believe it is an autoimmune disease, with a number of as-yet undetermined infectious, genetic or environmental causes or triggers. The inflammation of the bile duct may lead to infection, called cholangitis, when bile cannot flow through the duct.
Many people with early-stage PBC have no symptoms, but are diagnosed after routine blood tests indicate an abnormality of liver function, or an evaluation for another condition. Indeed, patients diagnosed with early-stage PBC may remain symptom-free for years, but catching it early means we can treat the condition and minimize liver damage.
Some patients with early PBC may experience fatigue, itching, and dry eyes and mouth. Patients in whom PBC has progressed may develop jaundice – a yellowing of the skin and eyes; digestive problems such as diarrhea and steatorrhea (greasy, foul-smelling stools); swollen feet (edema) and abdomen (ascites) from fluid buildup; cholesterol deposits (xanthomas) in the skin around the eyes, hands, elbows, knees, or buttocks; urinary tract infections; arthritis; thyroid problems; kidney stones and gallstones; and bone softening and fractures.
PBC may also cause a number of metabolic and autoimmune disorders, which include thyroid disease, which may appear long before PBC is diagnosed; rheumatoid arthritis; CREST syndrome, an immune disorder that is a type of scleroderma, a thickening of connective tissue which can occur in a number of locations, including the digestive tract, lungs, heart, blood vessels, and esophagus; and Raynaud's syndrome, a condition that affects small blood vessels in the hands and feet in response to cold or emotional stress.
Our gastrointestinal experts may perform a number of tests to determine if you have PBC, including:
For early PBC, doctors will prescribe the drug cholestyramine (Questran) to relieve itching, which works by reducing the level of bile acids in a patient's blood and, therefore, the skin. Doctors will also prescribe vitamin and mineral supplements to replace missing nutrients due to malabsorption. Our experts will also prescribe ursodeoxycholic acid, or UDCA, a naturally occurring bile acid to increase the flow of bile and thereby reduce inflammation of bile ducts in early stages of the disease. Side-effects of this drug may include diarrhea, aches, and trouble swallowing.
Our team of specialists will monitor all patients with PBC with regular examinations and blood tests to determine liver and bile duct function. If bile ducts require draining, our team can perform an endoscopic retrograde choangiopancreatography (ERCP).
In this procedure, which takes about an hour, patients, who fast for 12 hours beforehand, are given a sedative and a local anesthetic for the throat, either a spray or gargle, after which the physician places an endoscope into the mouth and guides it through the esophagus, stomach, and duodenum – the top of the small intestine. (The endoscope is a narrow, flexible tube equipped with a light and a camera).
The doctor then inserts a catheter (a smaller tube) through the endoscope and into the bile ducts. A contrast medium, or dye, is then injected through the catheter and an x-ray taken to visualize the ducts. If your physician finds that a duct is blocked, he or she can open it by angioplasty, a mechanical widening of a narrowed duct, and by inserting a tiny stent into the duct if necessary to keep it open.
Our team works together to prevent complications of PBC and maximize quality of life. When the disease is advanced, a liver transplant can help prolong life. Liver transplant are performed by surgeons in NewYork-Presbyterian's Center for Liver Disease and Transplantation (CLDT).
Advanced PBC may produce a number of serious conditions, which include liver failure and portal hypertension — increased pressure in the liver's portal vein, a major vein in the liver into which blood from the intestine, spleen, and pancreas flows. When scar tissue blocks normal blood flow through the liver and does not enable toxins to filter through the liver before entering the blood stream, blood backs up and increases pressure in the portal vein.
Other serious medical complications that may occur in advanced PBC include varices, or enlarged veins in the stomach and esophagus, and sometimes near the naval and rectum. Varices result from blocked portal vein circulation, which in turn causes blood to back up into other veins, producing bleeding, which requires immediate emergency care.
Patients with PBC may also have deficiencies of fat-soluble vitamins A, D, E, and K from malabsorption of nutrients due to liver damage. Osteoporosis or weak bones caused by a lack of vitamin D and calcium may occur for similar reasons — the liver is unable to process vitamin D and calcium. Cirrhosis is also a risk factor for liver, or hepatocellular cancer.