Primary sclerosing/biliary cholangitis

Question 1

Define primary biliary cholangitis

Answer 1

Autoimmune disease of the liver. Progressive destruction of the small bile ducts of the liver, causing bile and other toxins to build up (cholestasis)

Question 2

Pathology of primary biliary cholangitis

Answer 2

Interlobular bile duct damaged from chronic autoimmune granulotamous inflammation causing cholestasis -> cirrhosis, fibrosis, portal hypertension

Question 3

Cause of primary biliary cholangitis

Answer 3

Unknown environmental triggers (pollutants, xenobiotics, non-pathogenic bacteria) + genetic predisposition for non-tolerance to self-mitochondrial proteins

Question 4

Clinical manifestations of primary biliary cholangitis (5)

Answer 4

1. Often asymptomatic, chance finding on high ALP
2. Jaundice
3. Lethargy, sleepiness, pruritis
4. Skin pigmentation
5. Hepatosplenomegaly

Question 5

Investigations of primary biliary cholangitis (3)

Answer 5

1. Bloods - LFT - high ALP, mildly high ALT, AST, high bilirubin, low albumin
2. Ultrasound - look for extrahepatic cholestasis
3. Biopsy - not required but can look for granulomas around bile ducts

Question 6

Management of primary biliary cholangitis (4)

Answer 6

1. Symptomatic - try cholestyramine
2. Fat soluble vitamin prophylaxis
3. Monitoring - regular LFT and 2/year ultrasound
4. Liver transplant end disease

Question 7

Define primary sclerosing cholangitis

Answer 7

Progressive cholestasis with bile duct inflammation and strictures

Question 8

Clinical features of primary sclerosing cholangitis

Answer 8

1. Pruritis
2. Fatigue
3. Ascending cholangitis (if advanced)
4. Hepatic failure

Question 9

Investigations for primary sclerosing cholangitis

Answer 9

1. Bloods - high ALP, bilirubin, hypergammaglobulinaemia/IgM increase
2. AMA negative, ANCA, ANA, SMA positive
3. ERCP/MRCP
4. Liver biopsy - shows obliterative cholangitis

Question 10

Management of primary sclerosing cholangitis

Answer 10

1. Liver transplant
2. Yearly colonoscopy and ultrasound (bile duct, liver and colon cancers more common)
3. Cholecystectomy for gallbladder polyps

Question 11

Acute cholangitis symptoms

Answer 11

1. Continuous epigastric or RUQ pain
2. Vomiting
3. Fever
4. Local peritonism
5. Gallbladder mass

Question 12

Investigations for acute cholangitis

Answer 12

1. FBC - high WCC
2. Ultrasound - thick walled shrunked gallbladder
3. AXR - only shows 10% gallstones

Question 13

Treatment of acute cholangitis

Answer 13

1. NBM
2. Pain relief
3. IVI and antibiotics (e.g. co-amox)

Question 14

Symptoms of chronic cholangitis

Answer 14

1. Flatulent dyspepsia
2. Nausea
3. Vague abdominal discomfort
4. Distension
5. Flatulence
6. Fat intolerance

Question 15

Investigations for chronic cholangitis

Answer 15

1. Ultrasound - look for stones and assess CBD diameter

2. MRCP to find CBD stones`

Question 16

Treatment for chronic cholangitis

Answer 16

Cholecystectomy

Question 17

Describe biliary colic pain

Answer 17

Pain caused by gallstones obstructing a bile duct. Dull RUQ pain radiating to the back