Primary biliary cirrhosis (patient information)

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Primary Biliary Cirrhosis
Overview
What are the symptoms?
What are the causes?
Diagnosis
When to seek urgent medical care?
Treatment options
Where to find medical care for Primary biliary cirrhosis?
What to expect (Outlook/Prognosis)?
Possible complications
Primary biliary cirrhosis On the Web
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Images of Primary biliary cirrhosis
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FDA on Primary biliary cirrhosis
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Directions to Hospitals Treating Primary biliary cirrhosis
Risk calculators and risk factors for Primary biliary cirrhosis

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Aditya Govindavarjhulla, M.B.B.S. [2]

Overview

Primary biliary cirrhosis is irritation and swelling (inflammation) of the bile ducts of the liver, which blocks the flow of bile. This obstruction damages liver cells and leads to scarring called cirrhosis.

What are the symptoms of Primary biliary cirrhosis?

More than half of patients have no symptoms at the time of diagnosis. Symptoms usually come on gradually and may include:

• Abdominal pain
• Enlarged liver
• Fatigue
• Fatty deposits under the skin
• Fatty stools
• Itching
• Jaundice
• Soft yellow spots on the eyelid

What causes Primary biliary cirrhosis?

The cause of inflamed bile ducts in the liver is not known. However, primary biliary cirrhosis is an autoimmune disorder. That means your body's immune system mistakenly attacks healthy tissue.

The disease more commonly affects middle-aged women.

Long-term bile obstruction is believed to lead to liver cirrhosis. The disease may be associated with autoimmune disorders such as:

• Celiac disease
• Raynaud's phenomenon
• Sicca syndrome (dry eyes or mouth)
• Thyroid disease

Diagnosis

The doctor or nurse will perform a physical exam.

The following tests can check to see if your liver is working properly:

• Albumin blood test
• Liver function tests (serum alkaline phosphatase is most important)
• Prothrombin time (PT)
• Cholesterol and lipoprotein blood tests

Other tests that can help diagnose this disease are:

• Elevated immunoglobulin M level in the blood
• Liver biopsy
• Anti-mitochondrial antibodies (results are positive in about 95% of cases)

When to seek urgent medical care?

Call your health care provider if you have:

• Abdominal swelling
• Blood in the stools
• Confusion
• Jaundice
• Itching of the skin that does not go away and is not related to other causes
• Vomiting blood

Treatment options

The goal of treatment is to ease symptoms and prevent complications.

Cholestyramine (or colestipol) may reduce the itching. Ursodeoxycholic acid may improve removal of bile from the bloodstream may improve survival in some patients who have taken it for 4 years.

Vitamin replacement therapy restores vitamins A, K, and D, which are lost in fatty stools. A calcium supplement should be added to prevent or treat soft, weakened bones (osteomalacia).

Liver transplant before liver failure occurs may be successful.

Contraindicated medications

Primary bililary cirrhosis is considered an absolute contraindication to the use of the following medications:

• Fenofibrate
• Gemfibrozil

Where to find medical care for Primary biliary cirrhosis?

Directions to Hospitals Treating Primary biliary cirrhosis

What to expect (Outlook/Prognosis)?

The outcome can vary. If the condition is not treated, most patients will need a liver transplant to prevent death from this condition. About a quarter of patients who've had the disease for 10 years will experience liver failure. Doctors can now use a statistical model to predict the best time to do the transplant.

Possible complications

Progressive cirrhosis can lead to liver failure. Complications can include:

• Bleeding
• Damage to the brain (encephalopathy)
• Fluid and electrolyte imbalance
• Kidney failure
• Malabsorption
• Malnutrition
• Soft bones (osteomalacia)

Sources

National Library of Medicine