S2: Ch 3 GI 2-2

a. A chronic idiopathic progressive disease of intrahepatic and/or extrahepatic bile ducts characterized by thickening of bile duct walls and narrowing of their lumens; leads to cirrhosis, portal HTN, and liver failure.

a. There is a strong association w/UC (less so w/Crohn’s disease).
b. UC is present in 50-70% of pts w/PSC; often the UC may dominate the clinical picture.
c. Note: the course of PSC is unaffected by a colectomy done for UC.

a. S/S begin insidiously
b. Chronic cholestasis findings, including jaundice and pruritus; all pts eventually present w/chronic obstructive jaundice.
c. Other sx: fatigue, malaise, wt. loss.

a. ERCP and PTC are diagnostic studies of choice.
i. See multiple areas of bead-like structuring and bead-like dilatations of intrahepatic and extrahepatic ducts.
ii. Lab tests show cholestatic LFTs.

a. There is no curative tx other than liver transplantation.
b. When a dominant stricture causes cholestasis, ERCP w/stent placement for biliary drainage and bile duct dilatation may relieve symptoms.
c. Use cholestyramine for symptomatic relief (to decrease pruritus).

a. A chronic and progressive cholestatic liver disease characterized by destruction of intrahepatic bile ducts w/portal inflammation and scarring.
b. It is slowly progressive disease w/a variable course. It may progress to cirrhosis and end-stage liver failure.
c. It is an AUTOIMMUNE disease that is often associated w/other autoimmune disorders.

a. Middle-aged women.

a. Fatigue
b. Pruritus (early in course of disease)
c. Jaundice (late in course of disease)
d. RUQ discomfort
e. Xanthomata and Xanthelasmata
f. Osteoporosis
g. Portal HTN (w/resultant sequelae).

a. Antimitochondrial antibodies (AMAs).
b. This is the hallmark of the disease (specificity 98%).
c. If serum is positive for AMA, perform liver biopsy to confirm diagnosis.

a. Lab findings:
1. Cholestatic LFTs (elevated alk-P)
2. Antimitochondrial antibodies.
3. Elevated Cholesterol, HDL
4. Elevated immunoglobulin M
b. Liver biopsy (percutaneous or laparoscopic) to confirm dx.
c. Abdominal U/S or CT to R/O biliary obstruction.

a. Tx is symptomatic for pruritus (cholestyramine) and osteoporosis (Calcium, bisphosphonates, Vit D).
b. Ursodeoxycholic acid (a hydrophilic bile acid) has been shown to slow progression of the disease.

a. Liver transplantation

a. Tumour of intrahepatic or extrahepatic bile ducts
i. Most are adenocarcinomas.

a. 60s.

a. Proximal 1/3 off CBD (most common, also called Klatskin’s tumour)
b. Distal extrahepatic (best change of resectability).
c. Intrahepatic (least common)

a. Dismal. <1 yr after dx.

a. PSC is the major risk factor in the US. (1º sclerosing cholangitis).
b. Other risk factors:
1. UC
2. Choledochal cysts
3. Clonorchis sinensis infestation (in Hong Kong).

a. This disease occurs in response to chronic biliary obstruction from the following:
1. Long-standing mechanical obstruction
2. Sclerosing cholangitis
3. Cystic fibrosis
4. Biliary atresia.

a. Tumours in proximal 1/3 of CBD-involve the junction of right and left hepatic ducts.
b. Very poor prognosis bc they are unresectable.

a. Obstructive jaundice and associated sx: dark urine, clay-coloured stools, and pruritus.
b. Wt. loss.

a. Cholangiography (PTC or ERCP) for dx and assessment of resectability.
b. If the pt has an unresectable tumour (more likely the case w/proximal than distal bile duct tumours), stent placement is an option during either PTC or ERCP and may relieve biliary obstruction.

a. Most pts do not have resectable tumours at dx.
b. The survival rate is low despite aggressive chemo, stenting, or biliary draining.

a. Cystic dilations of biliary tree involving either the extrahepatic or intrahepatic ducts or both; more common in women (4:1).

a. Epigastric pain, jaundice, fever, and RUQ mass.

a. Cholangiocarcinoma (most feared complication: risk is about 20% over 20 yrs)
b. Hepatic abscess
c. Recurrent cholangitis/pancreatitis
d. Rupture
e. Biliary obstruction
f. Cirrhosis
g. Portal HTN.

a. U/S.

a. ERCP.

a. Surgery.
b. Complete resection of the cyst w/a biliary-enteric anastomosis to restore continuity of biliary system w/bowels.

a. Most common cause is iatrogenic injury (e.g., prior biliary surgery such as cholecystectomy, liver transplantation);
i. Other causes include:
1. Recurring choledocholithiasis
2. Chronic pancreatitis
3. PSC.

a. Obstructive Jaundice.