Primary biliary cirrhosis

A chronic, progressive autoimmune cholestatic liver disease characterized by immune-mediated destruction of small intrahepatic bile ducts (non-suppurative destructive cholangitis), resulting in impaired bile flow, progressive portal fibrosis, biliary cirrhosis, and eventual liver failure.

Causes

• Autoimmune-mediated attack against intrahepatic bile duct epithelial cells

  • Loss of immune tolerance against biliary epithelial antigens

  • Development of antimitochondrial antibodies (AMA)

• Genetic predisposition

  • Family history of autoimmune diseases

  • Genetic susceptibility (HLA associations)

• Environmental triggers

  • Cigarette smoking

  • Exposure to certain environmental chemicals

  • Xenobiotic exposure triggering immune responses

• Associated autoimmune diseases

  • Autoimmune thyroid disease

  • Sjögren syndrome

  • Rheumatoid arthritis

  • Systemic sclerosis (scleroderma)

  • Systemic lupus erythematosus (SLE)

  • Celiac disease

• Demographic associations

  • Female sex (strong predominance)

  • Middle age (commonly diagnosed between 40–60 years)

• Complications resulting from progressive disease

  • Cholestasis

  • Pruritus due to bile acid accumulation

  • Fat-soluble vitamin deficiency (A, D, E, K)

  • Osteoporosis/osteopenia

  • Portal hypertension

  • Esophageal varices

  • Ascites

  • Hepatic decompensation

  • Hepatocellular carcinoma in advanced cirrhosis

  • End-stage liver disease requiring liver transplantation