Primary biliary cirrhosis is an autoimmune liver disease that causes the slow degeneration of bile canaliculi, leading to cholestasis and cirrhosis. It affects women more than men and has no known cure. Symptoms include jaundice, fatigue, itching, and xanthoma. Medications can slow progression, and patients should take multivitamins and calcium supplements and avoid alcohol.
Primary biliary cirrhosis is an autoimmune liver disease characterized by the slow degeneration of the bile canaliculi, small tubes that collect bile, in the liver. As the disease progresses, it causes cholestasis, in which bile in the liver is unable to drain into the duodenum, the first portion of the small intestine, and bile builds up in the liver. The excess bile damages liver tissue, leading to cirrhosis or the replacement of healthy liver tissue with scar tissue and fibrous tissue.
Primary biliary cirrhosis affects about one in three to four thousand people, with women affected about nine times as often as men. There is a genetic predisposition, but the genetic basis of the disease is not fully understood. It is believed to be an autoimmune disease, caused by the immune system pathologically attacking enzymes normally produced by the liver, but the mechanism is not fully understood either. Risk factors for the disease have not been identified and there is no known cure. There is a possible link between primary biliary cirrhosis and gluten sensitivity, and the disease often accompanies other immune disorders such as rheumatoid arthritis or Sjögren’s syndrome.
Symptoms of primary biliary cirrhosis include jaundice or yellowing of the skin and eyes, fatigue, itching or itching, and xanthoma or cholesterol deposits in the skin. As cirrhosis develops, complications include ascites or fluid retention in the abdomen, dilated veins and bleeding in the esophagus, and splenomegaly or enlarged spleen. Hepatic encephalopathy, in which the patient experiences disorientation, irritability, coordination disturbances, amnesia, and possibly seizures and coma due to liver failure, may appear in the most severe cases. Primary biliary cirrhosis can be diagnosed by blood tests that check for signs of decreased liver function and certain antibodies, as well as by ultrasound, which can rule out bile duct blockage as a cause of cholestasis. The biopsy determines the stage of the disease.
There are four possible stages of primary biliary cirrhosis. In stage 1, portal stage, there is some inflammation of the hepatic portal vein, some damage to the bile duct, and often immune activity. Stage 2, periportal stage, is characterized by inflammation and fibrosis, or growth of fibrous tissue, around the hepatic portal vein. In stage 3, or septal stage, there is extensive growth of fibrous tissue and stage 4, biliary cirrhosis, is characterized by the presence of nodules, regenerative lumps that grow at the site of damaged tissue.
While there is no cure for primary biliary cirrhosis, medications like Ursodiol can help slow the progression of the disease by reducing the buildup of bile in the liver. Cholestyramine addressed itching by helping the body eliminate bile acids so they don’t re-enter the bloodstream. Patients with primary biliary cirrhosis are advised to take multivitamins and calcium supplements and abstain from alcohol.
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