Chronic Cholangitis Flashcards
Primary Biliary Cholangitis:
What occurs here?
How does it present?
Which conditions is it associated with?
How is it investigated?
How is it managed?
What are its potential complications?
➊ Autoimmune condition that only affects the INTRAhepatic ducts, resulting in obstructive jaundice and liver disease. Inflammation and damage to the bile ducts leads to cholestasis, back-pressure and resulting liver damage. With the outflow obstruction, bile acids, (conjugated) bilirubin, and cholesterol build up in the blood.
N.B. Bile acids help with the digestion of fats e.g. fat-soluble vitamins.
➋ Pts are often asymptomatic and incidentally found on LFTs. However, they may present with:
● Itching due to the raised bile acids - this is most commonly the only presenting symptom
● Jaundice due to raised bilirubin
● Obstructive jaundice (dark urine, pale stools)
● GI symptoms and abdominal pain due to lack of bile acids in gut
● Fatigue
● O/E - Xanthoma/Xanthelasma due to raised cholesterol
➌ ● Sjogren’s syndrome
● RA
● Systemic sclerosis
➍ ● LFTs - Raised ALP and GGT
● Anti-mitochondrial antibodies (AMA) - the most specific to PBC
➎ ● Ursodeoxycholic acid - This is a crucial part of treatment. It’s a non-toxic, hydrophilic bile acid that protects the cholangiocytes (biliary tree cells) from inflammation and damage, therefore slowing down disease progression, and improving survival.
● Colestyramine for itching (reduces intestinal absorption of bile acids)
● Replacement of fat-soluble vitamins (ADEK)
● Liver transplant in end-stage liver disease
➏ ● Cirrhosis (+ its related complications e.g. varices, portal htn)
● Fat-soluble vitamin deficiency (ADEK)
● Osteoporosis
● Hyperlipidaemia
Primary Sclerosing Cholangitis:
What occurs here?
How does it present?
Which conditions is it associated with?
How is it investigated?
How is it managed?
What are its potential complications?
➊ Autoimmine condition that affects both the INTRAhepatic and EXTRAhepatic, resulting in the formation of strictures and subsequent obstructive jaundice and liver disease.
➋ Pts are often asymptomatic and incidentally found on LFTs. However, they may present with:
● RUQ pain
● Itching due to the raised bile acids - this is most commonly the only presenting symptom
● Jaundice due to raised bilirubin
● Obstructive jaundice (dark urine, pale stools)
● GI symptoms and abdominal pain due to lack of bile acids in gut
● Fatigue
● Hepato/splenomegaly
➌ Ulcerative Colitis
➍ ● LFTs - Raised ALP and GGT
● MRCP - shows a beaded appearance
● Colonoscopy should be considered to assess for UC
➎ ● ERCP to dilate/stent dominant strictures
● Colestyramine for itching (reduces intestinal absorption of bile acids)
● Replacement of fat-soluble vitamins (ADEK)
● Liver transplant in end-stage liver disease
N.B. Ursodeoxycholic acid doesn’t have much benefit in PSC.
➏ ● Cholangiocarcinoma
● Cirrhosis (+ its related complications e.g. varices, portal htn)
● Fat-soluble vitamin deficiency (ADEK)
● Osteoporosis
