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Related Subjects: |Chronic liver disease |Cirrhosis |Alkaline phosphatase (ALP) |Liver Function Tests |Ascites Assessment and Management |Budd-Chiari syndrome |Autoimmune Hepatitis |Primary Biliary Cirrhosis |Primary Sclerosing Cholangitis |Wilson disease |Hereditary Haemochromatosis |Alpha-1 Antitrypsin (AAT) deficiency |Non alcoholic steatohepatitis (NASH) |Spontaneous Bacterial Peritonitis |Alcoholism and Alcoholic Liver Disease

PBC is associated with antimitochondrial antibodies found in 90-95% of cases with a specificity of 98% for this disease.

About

• Progressive autoimmune destruction of the small and medium-sized bile ducts
• There is cholestasis and eventual cirrhosis
• Positive Anti-mitochondrial antibody in 95%

Background

• A chronic progressive granulomatous inflammation of the liver
• Eventually leading to cholestasis, fibrosis and eventually cirrhosis.
• It is much more common in females and typically presents in middle-aged females.
• There is an association with other autoimmune diseases e.g. hypothyroidism.

Clinical

• Middle aged females classically
• Subclinical for years until it presents with signs of liver disease
• May be picked up incidentally on a blood test with a raised ALP
• Most commonly presents with lethargy and itch.
• Progressions with skin pigmentation, xanthoma, xanthelasma
• Jaundice is late and then hepatomegaly and splenomegaly can occur.
• There is malabsorption of ADEK fat-soluble vitamins

Investigations

• Raised Alkaline phosphatase (>1000) and GGT
• Raised Bilirubin and jaundice are elevated late
• Prolonged PTT - fat and Vitamin K malabsorption and a low albumin
• Nonspecific Raised IgM
• AST and ALT mildly elevated
• Hyperlipidaemia - not associated with increased risk of IHD **
• 98% have a Positive Anti-mitochondrial antibody M2 subtype (E2 fragment of Pyruvate dehydrogenase complex found on the inner mitochondrial membrane)
• USS shows no extrahepatic obstruction (Gallstones are common in this age and the population in general but in this case are likely irrelevant)
• Histology of liver biopsy: Granulomas are found around the proliferating bile ductules with liver fibrosis and cirrhosis but the poor prognostic value

Management

• Patients are treated with ursodeoxycholic acid which helps itch and possibly prognosis by increasing canalicular excretion of toxic bile acids and reducing intestinal bile acid reabsorption.
• No role for immunosuppression (steroids, colchicine, azathioprine and Methotrexate all failed)
• Patients are given vitamin replacement and nutrition is optimised.
• The onset of jaundice is a poor prognostic sign suggesting a prognosis of fewer than 2 years.
• Liver transplantation is an option and excellent results are seen.
• The complications are, portal hypertension and varices and eventual liver failure and hepatic encephalopathy

Poorer prognosis

• Raised bilirubin, Low albumin
• Prolonged prothrombin time, Oedema