Chronic Cholangitis

Primary Biliary Cholangitis (PBC)

This is an 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 subsequent liver damage. With the outflow obstruction, bile acids, (conjugated) bilirubin, and cholesterol build up in the blood instead.

N.B. Bile acids help with the digestion of fats e.g. fat-soluble vitamins.

Patients with PBC are often asymptomatic and the condition is 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

PBC is commonly associated with Rheumatic arthritis, Sjogren’s syndrome, and Systemic sclerosis.

Investigations:

• LFTs - Raised ALP and GGT

• Anti-mitochondrial antibodies (AMA) - the most specific to PBC

Management:

• 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

Complications:

• Cirrhosis (+ its related complications e.g. varices, portal hypertension)

• Fat-soluble vitamin deficiency (ADEK)

• Osteoporosis

• Hyperlipidaemia

Primary Sclerosing Cholangitis (PSC)

This is an autoimmune condition that affects both the INTRAhepatic and EXTRAhepatic, resulting in the formation of strictures and subsequent obstructive jaundice and liver disease.

Patients with PSC are often asymptomatic and the condition is 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

PSC is commonly associated with Ulcerative Colitis.

Investigations:

• LFTs - Raised ALP and GGT

• MRCP - shows a beaded appearance

• Colonoscopy should be considered to assess for UC

Management:

• Ursodeoxycholic acid - This doesn't improve survival with PSC, but does have a role in improving LFTs

• Colestyramine for itching (reduces intestinal absorption of bile acids)

• Replacement of fat-soluble vitamins (ADEK)

• Liver transplant in end-stage liver disease

Complications:

• Cholangiocarcinoma

• Cirrhosis (+ its related complications e.g. varices, portal hypetension)

• Fat-soluble vitamin deficiency (ADEK)

• Osteoporosis