Biliary cirrhosis

Overview

Primary biliary cirrhosis is an autoimmune disease of the liver marked by the slow progressive destruction of the small bile ducts (bile canaliculi) within the liver. When these ducts are damaged, bile builds up in the liver (cholestasis) and over time damages the tissue. This can lead to scarring, fibrosis and cirrhosis.It was previously thought to be a rare disease, but more recent studies have shown that it may affect up to 1 in 3-4,000 people; the sex ratio is at least 9:1 (women to men).

Symptoms

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

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

Diagnosis

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)