Biliary Cirrhosis and Cholangitis Flashcards
What is primary biliary cirrhosis?
Primary biliary cirrhosis is a condition where the immune system attacks the small bile ducts within the liver.
What is the pathophysiology of primary biliary cirrhosis?
The first parts to be affected are the intralobular ducts, also known as the Canals of Hering. This causes obstruction of the outflow of bile, which is called cholestasis. The back pressure of the bile obstruction and the overall disease process ultimately leads to fibrosis, cirrhosis, and liver failure.
Bile acids, bilirubin and cholesterol are usually excreted through the bile ducts into the intestines. When there is obstruction to the outflow of these chemicals they build up in the blood as they are not being excreted
What is the presentation of primary biliary cirrhosis?
-Fatigue
-Pruritus (bile acid)
-GI disturbances and abdominal pain
-Jaundice
-Pale Stools
-Xanthoma and xanthelasma (bile causes raised cholesterol)
-Signs of cirrhosis and failure
What other associations are often presented in individuals with primary biliary cirrhosis?
Middle-aged women
Other autoimmune diseases (thyroid, coeliac)
Rheumatoid conditions (eg systemic sclerosis, Sjogren’s and rheumatoid arthritis)
What would the liver function tests show for primary biliary cirrhosis?
Alkaline phosphatase would be raised
Other liver enzymes and bilirubin are raised in later disease
Anti-mitochondrial antibodies are the most specific to PBC
Anti-nuclear antibodies are present in about 35% of patients
What is the treatment option for primary biliary cirrhosis?
Ursodeoxycholic acid reduces the intestinal absorption of cholesterol
Colestyramine is a bile acid sequestrate in that it binds to bile acids to prevent absorption in the gut and can help with pruritus due to raised bile acids
What is primary sclerosis cholangitis?
Primary sclerosing cholangitis is a condition where the intrahepatic or extrahepatic ducts become strictured and fibrotic.
It causes obstruction to the flow of bile out of the liver and into the intestines, chronic bile obstruction eventually leads to liver inflammation (hepatitis), fibrosis, and cirrhosis.
What is the cause of primary sclerosis cholangitis?
The cause is mostly unclear although there is likely to be a combination of genetic, autoimmune, intestinal microbiome, and environmental factors.
There is an established association with ulcerative colitis - 70% of cases.
What is the presentation of primary sclerosing cholangitis?
-Fatigue
-Jaundice
-Prutitis
-RUQ pain
-Hepatomegaly
-Cirrhosis
What do the liver function tests show?
The liver function tests show a “cholestatic” picture.
ALP is the most deranged LFT.
There may also be a rise in bilirubin as the strictures become more severe and prevent bilirubin from being excreted through the bile duct.
What autoantibodies would be present on PSC test?
Arent very helpful but may indicate if there is an autoimmune element to the disease that may respond to immunosuppression.
Antineutrophil cytoplasmic antibody (p-ANCA) in up to 94%
Antinuclear antibodies (ANA) in up to 77%
Anticardiolipin antibodies (aCL) in up to 63%
What is the gold standard investigation for PSC diagnosis?
Magnetic resonance cholangiopancreatography.
This involves an MRI scan of the liver, bile ducts and pancreas. In primary sclerosis cholangitis it may show bile duct lesions or strictures.
What are the complications of PSC?
Acute bacterial cholangitis
Cholangiocarcinoma develops in 10-20% of cases
Colorectal cancer
Cirrhosis and liver failure
Biliary strictures
Fat soluble vitamin deficiencies
What is the management option for PSC?
- ERCP can be used to dilate and stent any strictures
- Colestyramine is a bile acid sequestrate in that it bind to bils acids to prevent absorption in the gut and can help with pruritus due to raised bile acids.
-Monitoring for complications (such as cholangiocarcinoma, cirrhoiss and oesphageal varices)
