Primary biliary cholangitis

PBC, Familial primary biliary cirrhosis, Primary Biliary Cirrhosis


Primary biliary cholangitis  is a chronic disease that affects the bile ducts within the liver. The bile ducts carry a fluid called bile from the liver to the gallbladder, where it is stored. The bile ducts become inflamed and damaged, which causes bile to build up in the liver. This abnormal buildup destroys liver tissue and in later stages results in cirrhosis. Primary biliary cirrhosis usually occurs between the ages of 40 and 60 and affects women more often than men. The cause of this condition is unknown, but research suggests that it is an autoimmune disease. Ursodiol is the only drug approved by the U.S. Food and Drug Administration for the treatment of primary biliary cirrhosis.

Symptoms - Primary biliary cholangitis

The following signs may be present in PBC:

  • Fatigue
  • Pruritus (itchy skin)
  • Jaundice (yellowing of the eyes and skin), due to increased bilirubin in the blood.
  • Xanthelasmata (focal collections of cholesterol in the skin, especially around the eyes)

Complications of cirrhosis and portal hypertension:

  • Fluid retention in the abdomen (ascites)
  • Hypersplenism
  • Esophageal varices
  • Hepatic encephalopathy, up to coma, in extreme cases.
  • Association with an extrahepatic autoimmune disorder such as Rheumatoid arthritis or Sjögren's syndrome (up to 80% incidence).

Causes - Primary biliary cholangitis

The importance of bile Bile is a greenish-yellow fluid produced in the cells of the liver. It's essential for digesting and absorbing fats and is one of the main avenues for eliminating drugs, cholesterol and metabolic byproducts from the body. Within the liver, bile is excreted into canal-like spaces between the liver cells, which drain into an interconnected series of thin tubes (ducts). The initial ducts are quite small, but become progressively larger as they spread through the liver, much like the branches of a tree. Bile leaves the liver through the common hepatic duct — the "trunk" of the biliary tree — and enters the gallbladder, a small, pear-shaped organ just below the liver, through the cystic duct. The gallbladder stores bile in a highly concentrated form until you eat, when it\'s released into the common bile duct. From there, bile flows to the upper part of the small intestine (duodenum), where it begins to break down the fat in food. A slow attack on your bile ducts

Prevention - Primary biliary cholangitis

Not supplied.

Diagnosis - Primary biliary cholangitis

  • To diagnose PBC, distinctions should be established from other conditions with similar symptoms, such as autoimmune hepatitis or primary sclerosing cholangitis (PSC). Diagnostic blood tests include:
  • Deranged liver function tests (high alkaline phosphatase, elevated AST, ALT) 
  • Presence of certain antibodies: antimitochondrial antibody, antinuclear antibody (the M2-IgG antimitochondrial antibody is the most specific test)

Prognosis - Primary biliary cholangitis

Not supplied.

Treatment - Primary biliary cholangitis

Ursodiol is the drug approved by the FDA for the treatment of primary biliary cholangitis.

Resources - Primary biliary cholangitis

  • NIH
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