What Causes Primary Biliary Cirrhosis?

The cause of PBC is unknown. It is most likely an autoimmune disease, where the body's immune system attacks its own cells. In this disease, the bile ducts are under attack and are destroyed.

What Are The Symptoms?

Although some people with primary biliary cirrhosis remain symptom-free for years after they're diagnosed, others experience a number of signs and symptoms. Common early symptoms include:

• Fatigue
• Itchy skin
• Dry eyes and mouth

Later signs and symptoms may include:

• Pain in the upper right portion of the abdomen
• Bone, muscle or joint (musculoskeletal) pain
• Yellowing of the skin and eyes (jaundice)
• Darkening of the skin that's not related to sun exposure (hyper pigmentation)
• Swollen feet and ankles (edema)

The Immune System Response

The inflammation of primary biliary cirrhosis begins when T lymphocytes (T cells) start accumulating in your liver. Normally, T cells recognize and help defend against harmful invaders, such as bacteria. But in primary biliary cirrhosis, the T cells invade and destroy the cells lining the small bile ducts in your liver. Inflammation in the smallest ducts spreads, in time, and destroys nearby liver cells. As these cells are destroyed, they're replaced by scar tissue (fibrosis) that can contribute to cirrhosis. Cirrhosis is scarring of liver tissue that makes it difficult for your liver to carry out essential functions. (Mayo Clinic)

How is PBC Diagnosed?

Diagnosis is made with special blood tests including liver bichemistry. One such test detects the presence of antimitochondrial antibodies (AMA) in blood. Also, a liver biopsy may be performed. This is a procedure in which a small needle is inserted into the liver to take a sample of the tissue for analysis. (AARDA)

The Different Stages of PBC.

There are 4 stages of PBC.

• Stage 1 (portal stage of Ludwig): Portal inflammation, bile duct abnormalities, or both are present.
• Stage 2 (periportal stage): Periportal fibrosis is present, with or without periportal inflammation or prominent enlargement of the portal tracts with seemingly intact, newly formed limiting plates.
• Stage 3 (septal stage): Septal fibrosis with active inflammatory, passive paucicellular septa, or both are present.
• Stage 4 (cirrhosis): Nodules with various degrees of inflammation are present (Medscape)

What Is the Treatment for PBC?

Patients most often take a daily dose of a drug called ursodiol. This is urseodeoxycholic acid, a naturally occurring bile acid. This drug improves the liver’s ability to function in PBC patients. It also extends life expectancy and may delay the need for a liver transplant. The recommended dose is 15 mg per kilogram of body weight, and the medication must be taken every day for life, or until a liver transplant. Patients rarely experience side effects from ursodiol. Other drugs are effective at relieving the symptoms of PBC, including the itching.

Liver transplant: When treatments no longer control primary biliary cirrhosis and the liver begins to fail, a liver transplant may help prolong life. A liver transplant is a procedure to remove your diseased liver and replace it with a healthy liver from a donor. Primary biliary cirrhosis often recurs in the transplanted liver, but it may take several years to develop. People with primary biliary cirrhosis who have liver transplants generally have high survival rates (Mayoclinic)

How Can PBC Be Prevented?

Since the cause of the disease is not known, it is not possible to prevent PBC.

Work Citied

1. Mayoclinic.org. http://www.mayoclinic.org/diseases-conditions/primary-biliary-cirrhosis/basics/definition/con-20029377
2. American Autoimmune Related Disease Association. https://www.aarda.org/diseaseinfo/primary-biliary-cirrhosis/
3. American Liver Foundation. http://www.liverfoundation.org/abouttheliver/info/pbc/
4. Medscape. http://emedicine.medscape.com/article/171117-overview
5. NATURE REVIEWS GASTROENTEROLOGY & HEPATOLOGY. http://www.nature.com/nrgastro/journal/v12/n3/full/nrgastro.2015.12.html