| Chronic liver disease
| 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.
- Progressive autoimmune destruction of the small and medium-sized bile ducts
- There is cholestasis and eventual cirrhosis
- Positive Anti-mitochondrial antibody in 95%
- 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.
- 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
- Raised Alkaline phosphatase (>1000) and GGT
- Raised Bilirubin and jaundice are elevated late
- Prolonged PTT - fat and Vitamin K malabsorption and a low albumin
- Non specific 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
- 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
- Raised bilirubin, Low albumin
- Prolonged prothrombin time, Oedema