Cholestatic & AI Liver Dz

Question 1

mechanism and etiology of primary biliary cirrhosis

Answer 1

autoimmune dz of unknown etiology

Question 2

pathogenesis of PBC

Answer 2

destruction of small intrahepatic bile ducts leads to cholestasis (impaired bile flow)

Question 3

what are the long-term consequences of cholestasis?

Answer 3

high concentrations of potentially toxic bile constituents, leading to progressive liver dz

Question 4

epidemiology of PBC

Answer 4

95% women, usually 40-60 yrs, rare

Question 5

PBC is associated with what autoimmune diseases?

Answer 5

thyroid dz, sjogren’s syndrome

Question 6

most common PBC presentation

Answer 6

asymptomatic

Question 7

signs of PBC

Answer 7

fatigue, pruritis, jaundice, hepatomegaly, splenomegaly, xanthelasma

Question 8

lab dx of PBC

Answer 8

especially high AP, antimitochondrial antibody (AMA), sometimes increased serum IgM

Question 9

what would you find on a liver biopsy for PBC?

Answer 9

granulomas, florid bile duct lesion (lymphocytic infiltrate and attenuated ductule epithelium)

Question 10

how can you exclude small duct PCS from the differential?

Answer 10

evalulate biliary tree for obstruction

Question 11

name two major complications of PBC (other than liver dz)

Answer 11

decreased bile acids –> fat malabsorption, weight loss, and steatorrhea; portal HTN (obviously also liver dz)

Question 12

only drug approved for tx of PBC

Answer 12

ursodiol (hydrophobic bile acid) improves survival and delay progression if given early

Question 13

in addition to ursodiol, what should be given to pts with PBC?

Answer 13

vitamins and calcium to px bone dz; cholestyramine for pruritis (bile acid sequestrant?)

Question 14

should a pt with PBC ever receive a liver transplant?

Answer 14

yes, if there is advanced dz

Question 15

pathogenesis of primary sclerosing cholangitis

Answer 15

inflammation and sclerosis leads to obliteration of medium/large biliary ducts, causing cholestasis

Question 16

epidemiology of PSC

Answer 16

predominantly males, usually 20-40 at dx, less rare than PBC but still rare

Question 17

there is a strong association between PSC and ____

Answer 17

inflammatory bowel disease (ulcerative cholitis mainly)

Question 18

patient presentation: PSC

Answer 18

asymptomatic 15-40%, fatigue, pruritis, abdominal pain, jaundice, fever, hepatosplenomegaly

Question 19

lab dx of PSC is less helpful than in PBC, but may show?

Answer 19

especially high AP, p-ANCA (80%), hyperIgG, other autoantibodies

Question 20

what is the best way to dx PSC (2 answers)

Answer 20

cholangiogram (ERCP) for “beads on a string” or liver biopsy for “onion-skin bile ducts”

Question 21

secondary causes of sclerosing cholangitis (must always be ruled out)

Answer 21

drugs, bacterial, gallstones, bile duct cancers/strictures, AIDS-assoc cholangiopathy

Question 22

complications of PSC

Answer 22

portal HTN, progressive liver dz, recurrent bacteremia (cholangitis), cholangiocarcinoma (10-15% lifetime), colon cancer (if UC also)

Question 23

management of PSC

Answer 23

medical tx doesn’t help!, dilation and stent of dominant strictures (ERCP), aggressive abx for cholangitis, liver transplant

Question 24

presentation of autoimmune hepatitis

Answer 24

fatigue, malaise, nausea, abdominal pain, pruritis, jaundice, hepatomegaly, arthralgias, chronic liver dz, fulminant hepatitis

Question 25

autoimmune diseases assoc with AIH

Answer 25

thyroiditis, rheumatologic (arthritis, sjogren)

Question 26

which gender usually gets AIH?

Answer 26

women (75-80%)

Question 27

lab diagnosis of AIH

Answer 27

first rule out viral hepatitis (looks just like it!), elevated AST/ALT predominantly, elevated ANA or other autoimmune ab, elevated immunoglobulins

Question 28

biopsy dx of AIH

Answer 28

interface hepatitis and plasma cells (don’t have to have plasma cells)

Question 29

type I AIH is positive for what autoantibodies?

Answer 29

ANA, ASMA

Question 30

type II AIH is positive for what autoantibodies?

Answer 30

Anti-LKM

Question 31

management of AIH

Answer 31

1. immunosuppression (prednisone, azathioprine), 2. if refractory, cyclosporine, tacrolimus, MPM, 3. liver transplant