Primary biliary cirrhosis (PBC)

Primary biliary cirrhosis - the

M

rule

• IgM (elevated in advanced state)

• anti-M, M2 subtype

  itochondrial antibodies

• Middle aged females (female to male 9:1)

strong association with other autoimmune diseases, such as Sjögren syndrome, RA, and scleroderma most common symptoms = fatigue and pruritus 1/3 may be asymptomatic

Complications

• malabsorption: osteomalacia, hypothyroidism, coagulopathy; xanthoma & xanthelasma (2° fat-soluble vitamins)
• sicca syndrome occurs in 70% of cases
• portal hypertension: ascites, variceal haemorrhage
• hepatocellular cancer (20-fold increased risk)

Dx Liver biopsy is the most accurate test.

N.B. Vanishing bile duct syndrome, a rare disease invloving progressive destruction of the intrahepatic bile ducts has same pattern of liver injury.

Histological hallmark = ductopenia; periductal mononuclear inflammatory infiltrate with bile duct destruction

PBC is the most common cause of ductopenia in adults (other causes = failing liver transplantation, Hodgkin's disease, GVHD, sarcoid, CMV, infection, HIV and medication toxicity)

In the precirrhotic stage, the interlobular bile ducts are destroyed by granulomatous inflammation ("florid duct lesion") and a heavy portal tract infiltrate of macrophages, lymphocytes (majority), plasma cells, and eosinophils is present.

Lab: *

normal bilirubin level with elevated ALPase and GGT