Cholestatic & AI Liver Dz Flashcards Preview

Gastrointestinal > Cholestatic & AI Liver Dz > Flashcards

Flashcards in Cholestatic & AI Liver Dz Deck (31):
1

mechanism and etiology of primary biliary cirrhosis

autoimmune dz of unknown etiology

2

pathogenesis of PBC

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

3

what are the long-term consequences of cholestasis?

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

4

epidemiology of PBC

95% women, usually 40-60 yrs, rare

5

PBC is associated with what autoimmune diseases?

thyroid dz, sjogren's syndrome

6

most common PBC presentation

asymptomatic

7

signs of PBC

fatigue, pruritis, jaundice, hepatomegaly, splenomegaly, xanthelasma

8

lab dx of PBC

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

9

what would you find on a liver biopsy for PBC?

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

10

how can you exclude small duct PCS from the differential?

evalulate biliary tree for obstruction

11

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

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

12

only drug approved for tx of PBC

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

13

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

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

14

should a pt with PBC ever receive a liver transplant?

yes, if there is advanced dz

15

pathogenesis of primary sclerosing cholangitis

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

16

epidemiology of PSC

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

17

there is a strong association between PSC and ____

inflammatory bowel disease (ulcerative cholitis mainly)

18

patient presentation: PSC

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

19

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

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

20

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

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

21

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

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

22

complications of PSC

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

23

management of PSC

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

24

presentation of autoimmune hepatitis

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

25

autoimmune diseases assoc with AIH

thyroiditis, rheumatologic (arthritis, sjogren)

26

which gender usually gets AIH?

women (75-80%)

27

lab diagnosis of AIH

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

28

biopsy dx of AIH

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

29

type I AIH is positive for what autoantibodies?

ANA, ASMA

30

type II AIH is positive for what autoantibodies?

Anti-LKM

31

management of AIH

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