Primary biliary cirrhosis refers to a condition in which the bile ducts are obstructed due to chronic inflammation. Early diagnosis of this debilitating liver disease is the thumb rule for getting good prognosis.
Primary biliary cirrhosis (PBC) is an ailment of the liver organ, which is characterized by inflammation of the bile ducts. In a patient diagnosed with this chronic disease, the inflamed bile ducts are unable to function properly and collect bile juice secreted by the liver. In due course of time, bile builds up in the liver causing irreversible damage to the tissues. If left untreated, the damaged tissues and accumulated bile juice (a condition called cholestasis) result in progressive tissue scarring and liver cirrhosis.
Causes
The actual cause of primary biliary cirrhosis is nor clear in medical science. As per the studies that have been conducted so far, this chronic ailment is shown to be linked with autoimmune disorder. Those who are genetically predisposed to this disease are at a high risk of developing it after exposure to infections and alike triggers. In many instances, primary biliary cirrhosis runs in families, and those with affected siblings and parents are more susceptible to this liver disease than others. In addition to PBC, patients may be afflicted with rheumatoid arthritis or other extrahepatic autoimmune diseases.
Symptoms
The occurrence rate of primary biliary cirrhosis is low as compared to other autoimmune diseases. As per estimation, one out of 4,000 people are diagnosed with this liver disease. It may affect both gender and people of all age groups, but majority of the patients are women who are 40 years and older. In the first onset of disease, the immune system mistakenly targets cells that line bile canaliculi. Manifested primary biliary cirrhosis symptoms include the following.
- Increased weakness
- Skin itchiness that worsen at night
- Dry mouth and eyes
- Pain in the upper right abdomen
- Increased redness of palms
- Throwing up blood
- Joint and bone pain
- Edema in ankles and belly
- Elevated bilirubin level in blood
- Yellowish coloration of skin and eyes (jaundice)
- High cholesterol level
- Deposition of cholesterol under the skin (xanthoma)
Treatment
Fortunately most patients have mild tissue scarring at the time of diagnosis. In about 50 percent patients, disease identification is done while performing blood works for routine health checkup. Blood test results that signify blockage of bile ducts are reported, such as the presence of antimitochondrial antibody (AMA) in the bloodstream. To rule out other liver conditions, the doctor may perform imaging tests of the liver, ERCP of bile ducts (endoscopic retrograde cholangiopancreatography) and liver biopsy.
Timely treatment of primary biliary cirrhosis with prescription medications is effectual to slow down tissue damage and scarring. Therapeutic intervention is focused on reducing the speed of tissue injury along with management of signs and symptoms. Most likely, the patient will be prescribed with medications that contain ursodeoxycholic acid as an active component. This acid is naturally found in bile juice and its function is related to minimizing cholesterol secretion.
In addition, specific medicines are recommended based on the patient’s symptoms. Say for instance, antihistamine tablets are effective for treating skin itchiness associated with primary biliary cirrhosis. In the same way, administration of immunosuppressant drugs and corticosteroids are aimed at counteracting the hypersensitive responses of the immune system. As an approach to lead a quality life, a patient should perform regular exercise, eat well-planned diet and take dietary supplements.
Once primary biliary cirrhosis is confirmed and treatment is started, the doctor may suggest alcohol abstinence and reduction of fatty foods, which otherwise cause a nauseated feeling. Even taking small quantities of alcoholic beverages will worsen scarring of liver tissues. In some incidences, patients experience liver failure complications, requiring surgery for liver transplant. However, this condition may reappear in people who have undergone this surgical procedure. Learn more on cirrhosis prognosis.
If primary biliary cirrhosis is diagnosed early and correct treatment approaches are practiced, the disease prognosis is generally good. Nevertheless, the rate of disease progression varies significantly from one patient to another. While some show notable symptoms within 2 years period, others may report medical problems related to PBC after 10 years. The estimated life expectancy averages 10 years, if diagnosis is made after symptoms are manifested.
