Primary sclerosing cholangitis, primary biliary cirrhosis Flashcards Preview

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Flashcards in Primary sclerosing cholangitis, primary biliary cirrhosis Deck (28):
1

Define primary sclerosing cholangitis

Bile duct injury ( intra and extrahepatic) characterized by inflammation, fibrosis, and strictures

2

In what population is primary sclerosing cholangitis most frequently seen?

Men, usually before 45

3

Primary sclerosing cholangitis is an ______ disease

autoimmune
thought that an inflammatory/ infectious trigger initiates (bacteria from portal circulation??)
p-ANCA

4

______ attack on the bile duct leads to scarring and strictures in PSC

T cell

5

75% of people with PSC have ________

ulcerative colitis

6

Describe the clinical presentation of PSC

asymptomatic in 10% of people
fatigue, abdominal pain, weight loss, pruritis, episodic jaundice, rarely acute cholangitis

7

How is PSC diagnosed?

Alkaline phosphatase 2x normal
Elevated transaminases, bilirubin, prothrombin time
low albumin
ERCP is gold standard for diagnosis: multifocal strictures with intervening dilated ducts (beaded appearance)
onion ring appearance on liver biopsy

8

Describe the prognosis of PSC

Slow progression to secondary biliary cirrhosis, portal HTN, liver failure
12 yr median survival
increased risk of cholangiocarcinoma

9

Describe treatment of PSC

supportive- no intervention is successful in slowing disease progression (including immune suppression)
Endoscopic procedures to remove stones, stent strictures
liver transplant (new liver will get PSC)

10

_______ is the most common form of cholangiocarcinoma

Klatskin tumor (at bifurcation)

11

List causes that have been associated with cholangiocarcinoma

PSC, gallstones, liver flukes, cysts, toxic exposure

12

Describe the presentation of cholangiocarcinoma

non-specific weight loss, anorexia, abdominal pain, failure to thrive
new onset or worsening LFT elevation, jaundice

13

What are serum tumor markers for cholangiocarcinoma

CEA and CA 19-9

14

Cholangiocarcinoma is extremely difficult to diagnose particularly with longstanding _______

PSC

15

What is the prognosis for cholangiocarcinoma?

Extremely poor- 3 years if tumor is resectable, 1 year if not

16

Define primary biliary cirrhosis

Progressive chronic cholestatic autoimmune disease that affects the small microscopic intrahepatic bile ducts

17

What population is most commonly affected by primary biliary cirrhosis?

women>>men
6th or 7th decade

18

What immunologic abnormalities are seen in PBC?

T-cell mediated autoimmune response against bile duct epithelial cells

presence of antimitochondrial antibodies (AMA), IgM hypergammaglobulinemia, circulating immune complexes

19

Describe the pathophysiology of PBC

Chronic damage/destruction of small ducts causes obstruction of bile flow and cholestasis>>>hepatocyte injury, scarring + accumulation of bile acids

Possible cirrhosis and liver failure

20

What are symptoms of PBC?

Very often asymptomatic at diagnosis

fatigue, pruritis worse at night, jaundice

21

What is the underlying cause of the bone disease seen in PBC?

Bone disease – osteoporosis and osteomalacia secondary to Vit D malabsorption

22

Describe the metabolic sequelae of PBC

Weight loss, steatorrhea, bone disease, vitamin deficiencies – bile secretory failure and low intestinal bile salt levels

23

Describe the liver sequelae of PBC

Later stages- cirrhosis, portal hypertension
Ascites
Esophageal varices and bleeding
Increased risk of hepatocellular carcinoma

24

How is PBC diagnosed?

Elevated ALKP, bilirubin
Positive AMA, hypergammaglobulinemia
Liver biopsy shows patchy destruction of interlobular bile ducts with mononuclear infiltrate and possible granulomas

25

Describe the prognosis of PBC

Variable with wide spectrum
Generally slowly progressive disease and patients can be asymptomatic for years

Asymptomatic – normal life span
Symptomatic – median 11-12 year survival
Bilirubin greatest predictor of survival
Jaundice as marker of disease progression, cirrhosis

26

______ is the greatest prognostic marker in PBC

Bilirubin

27

In PBC, ______ is a marker of disease progression and possible cirrhosis

Jaundice

28

How is PBC treated

UDCA: cytoprotectvie effect, inhibits absorption of toxic bile salts, improvement in liver biochemistry, improved survival free of transplant

Supportive care: treat pruritis, vitamin deficiencies, liver transplant once ESLD