Primary Biliary Cirrhosis

Primary Biliary Cirrhosis (PBC) is a rare liver disease that affects 1 in every 100,000 people. PBC causes scarring of the liver which results in scar tissue formation and fibrosis. Fibrosis leads to loss of hepatic function and eventually death from complications such as cirrhosis or liver cancer. There are no effective treatments for PBC; however, there are several medications that have been used successfully to treat it.

Symptoms of Primary Biliary Cirrhosis

The most common symptom of PBC is jaundice (yellowing of the skin and eyes). Other symptoms include:

Fatigue or weakness. Some people may not feel tired at all, while others experience fatigue that persists into later stages of the disease.

Weight loss. Weight gain occurs in some cases due to increased appetite and decreased activity level.

Nausea or vomiting.

Abdominal pain.

Loss of hair. Hair loss may occur in some cases, especially if the disease progresses rapidly. Lymph nodes may become enlarged and swollen. Liver transplantation is sometimes necessary to save the patient’s life.

PBC may also increase the risk of developing blood clots, which can lead to stroke or heart attack.

Diagnosing Primary Biliary Cirrhosis

The following procedures may be used to diagnose PBC:

Blood tests. These help detect decreased numbers of certain blood cells, such as red blood cells. The levels of certain liver enzymes may also be high in people with PBC.

Liver biopsy. A procedure in which a small sample of liver tissue is removed and examined under a microscope. A liver biopsy may be necessary to confirm a diagnosis of PBC.

Treating Primary Biliary Cirrhosis

Effective treatments for PBC are currently unavailable, but several drugs have been used with varying results. It is important for those who have PBC to eat a healthy diet, get plenty of rest, and avoid alcohol and certain medications.

The following drugs have been used to treat PBC:

1) ursodeoxycholic acid (UDCA).

Also called Actigall, this medication helps suppress the immune system and reduces inflammation. It is usually effective in slowing down the progression of PBC in most people. A liver transplant may be necessary in some cases where other treatments have failed to improve liver damage.

2) corticosteroids.

These include prednisone and prednisolone. They help suppress the body’s immune system and are effective in treating PBC in some people.

3) tacrolismus (TAC).

This drug may suppress the immune response in some people with PBC who have overactive immune systems. TAC is sometimes effective in slowing down disease progression.

4) mycophenolate mofetil (CellCept).

This medication suppresses the body’s immune system and has been used to treat PBC. It is usually taken for a period of three months.

The symptoms of PBC are very similar to those of primary sclerosing cholangitis (PSC), a related liver disease that also causes scarring of the bile ducts. It is important to see a doctor at the first sign of symptoms, to avoid further liver damage.

The prognosis for PBC is poor. Most people with the disease will experience liver failure and die within 15 years after the disease is diagnosed. However, liver transplantation has enabled some people to live for several years after receiving a new liver.

Possible Complications

Complications of PBC may include:

Liver failure. This occurs when the liver is so damaged that it can no longer function properly. A liver transplant may reverse liver failure, but the disease often recurs in the transplanted liver.

Blood clots. These may cause stroke or heart attack.

If left untreated, PBC may increase the risk of developing cancer of the bile ducts.

“I was always tired and drained, but I just thought that was how life was. Then, when I was 31 years old, I started getting these strange lumps all over my body. It turns out that I had Primary Biliary Cirrhosis. It was a huge shock.”

-Helen B. (35 years old)

Reviewed by: Joanna Mak

Also reviewed by: National Institute of Health (April 2005)

Recent Research (PubMed.

Sources & references used in this article:

Primary biliary cirrhosis by MM Kaplan – New England Journal of Medicine, 1996 – Mass Medical Soc

Primary biliary cirrhosis by EH Ahrens Jr, MA Payne, HG Kunkel… – Medicine, 1950 – journals.lww.com

Management of primary biliary cirrhosis by EJ Heathcote – Hepatology, 2000 – Wiley Online Library

Primary biliary cirrhosis by MM Kaplan – New England Journal of Medicine, 1987 – Mass Medical Soc