INTRAHEPATIC BILIARY

DISORDERS
Intrahepatic biliary diseases disrupts the flow of bile
through the liver, causing cholestasis and biliary
cirrhosis. Among the causes of intrahepatic biliary
disease are primary biliary, primary sclerosing
cholangitis, and secondary biliary cirrhosis.
PRIMARY BILIARY CIRRHOSIS
Involves inflammation and scarring of small
intrahepatic bile ducts, portal inflammation, and
progressive scarring of liver tissue.
Mostly seen in women 30 to 65 years of age and
accounts for 2% to 5% of cases of cirrhosis
Up to 84% of persons with primary biliary cirrhosis
have at least one other autoimmune disorder, such as
sclerodema, Hashimoto’s thyroiditis, rheumatoid
arthritis, or Sjogren’s syndrome
Presumed to be autoimmune in nature
Liver transplant is the only known treatment of this
disease
Why it is considered autoimmune?
abnormalities of the humoral and cellular immune
systems
multiple circulating autoantibodies
granulomas in the liver and regional lymph nodes
impaired regulation of both B and T lymphocytes
the association of this disease with a variety of
autoimmune-mediated diseases 
Symptoms
Puritis
Weight loss
Fatigue
Dark Urine
Pale stools
Jaundice
PRIMARY SCLEROSING
CHOLANGITIS
Involves inflammation of hepatic bile ducts.
Chronic cholestasis disease of unknown origin that
causes destruction and fibrosis of intrahepatic and
extrahepatic bile ducts.
Associated with inflammatory bowel disease
Often seen in men from 30 to 50 years of age
The only treatment is liver transplant
Symptoms
Fatigue
Jaundice
Pruritis
Later signs:
Cirrhosis
Portal hypertension
Liver failure
Secondary Biliary Cirrhosis
Seconday Biilary Cirrhosis from prolonged
obstruction of the extrabiliary tree.
Other causes are malignant neoplasms of the biliary
tree or head of the pancreas and structures of the
common duct caused by previous surgical procedures.