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Primary biliary cirrhosis (PBC): Causes, Signs, Symptoms and Treatment

 


Primary biliary cirrhosis (PBC) is a progressive liver disease characterized by bile accumulation (cholestasis) that causes damage to the small bile ducts that drain bile from the liver. This pressure buildup eventually damages the bile ducts, causing liver cell destruction. Cirrhosis and liver failure develop as the disease advances and enough liver cells die. Bile is produced in the liver and then delivered via the bile ducts to the gallbladder and gut, where it aids in the digestion of fats and fat-soluble vitamins (A,D,E,K). When bile is unable to be emptied from the liver, it causes inflammation and cell death. Scar tissue progressively replaces the areas of the damaged liver, and the body is unable to execute essential processes.

Causes of primary biliary cirrhosis

PBC has an unclear cause. It is most likely an autoimmune disease, in which the immune system assaults the body's own cells. The bile ducts are attacked and damaged in this condition.

Risk factors for primary biliary cirrhosis

Because a person with PBC is likely to have a family member who also has the condition, there may be a genetic component to its development.

Women are nine times more likely than men to develop PBC. It most frequently develops in the middle age between the ages of 40 to 60.

Signs and symptoms for primary biliary cirrhosis

The most common early symptoms are exhaustion or a feeling of being unnaturally tired, as well as itchy skin. The skin may get darkened and discolored in some spots as a result of the acute itching and scratching. Some patients may have right upper quadrant stomach pain, which is where the liver is located, as well as dry mouth and eyes.

Cirrhosis symptoms may arise if the liver damage worsens. These symptoms include muscular atrophy, ascites (abdominal swelling caused by fluid buildup), leg edema, jaundice (a yellow tint of the skin), and disorientation.

Diagnosis of primary biliary cirrhosis

Primary biliary cirrhosis is considered when the doctor suspects that there is liver inflammation based on the history and physical examination. There may be a few symptoms at first, such as itching or exhaustion, to assist lead additional tests.  When a diagnosis of antimitochondrial antibody (AMA) is suspected, a blood test is routinely performed. This is true for more than 90% of PBC patients. Other blood tests, such as a complete blood count (CBC) and cholesterol readings, may be explored. Ultrasound, CT scan, or MRI imaging of the abdomen is frequently used to examine for structural abnormalities in the liver and tumors.

 

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