Cholestatic & AI Liver Dz Flashcards
mechanism and etiology of primary biliary cirrhosis
autoimmune dz of unknown etiology
pathogenesis of PBC
destruction of small intrahepatic bile ducts leads to cholestasis (impaired bile flow)
what are the long-term consequences of cholestasis?
high concentrations of potentially toxic bile constituents, leading to progressive liver dz
epidemiology of PBC
95% women, usually 40-60 yrs, rare
PBC is associated with what autoimmune diseases?
thyroid dz, sjogren’s syndrome
most common PBC presentation
asymptomatic
signs of PBC
fatigue, pruritis, jaundice, hepatomegaly, splenomegaly, xanthelasma
lab dx of PBC
especially high AP, antimitochondrial antibody (AMA), sometimes increased serum IgM
what would you find on a liver biopsy for PBC?
granulomas, florid bile duct lesion (lymphocytic infiltrate and attenuated ductule epithelium)
how can you exclude small duct PCS from the differential?
evalulate biliary tree for obstruction
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)
only drug approved for tx of PBC
ursodiol (hydrophobic bile acid) improves survival and delay progression if given early
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?)
should a pt with PBC ever receive a liver transplant?
yes, if there is advanced dz
pathogenesis of primary sclerosing cholangitis
inflammation and sclerosis leads to obliteration of medium/large biliary ducts, causing cholestasis
epidemiology of PSC
predominantly males, usually 20-40 at dx, less rare than PBC but still rare
there is a strong association between PSC and ____
inflammatory bowel disease (ulcerative cholitis mainly)
patient presentation: PSC
asymptomatic 15-40%, fatigue, pruritis, abdominal pain, jaundice, fever, hepatosplenomegaly
lab dx of PSC is less helpful than in PBC, but may show?
especially high AP, p-ANCA (80%), hyperIgG, other autoantibodies
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”
secondary causes of sclerosing cholangitis (must always be ruled out)
drugs, bacterial, gallstones, bile duct cancers/strictures, AIDS-assoc cholangiopathy
complications of PSC
portal HTN, progressive liver dz, recurrent bacteremia (cholangitis), cholangiocarcinoma (10-15% lifetime), colon cancer (if UC also)
management of PSC
medical tx doesn’t help!, dilation and stent of dominant strictures (ERCP), aggressive abx for cholangitis, liver transplant
presentation of autoimmune hepatitis
fatigue, malaise, nausea, abdominal pain, pruritis, jaundice, hepatomegaly, arthralgias, chronic liver dz, fulminant hepatitis
autoimmune diseases assoc with AIH
thyroiditis, rheumatologic (arthritis, sjogren)
which gender usually gets AIH?
women (75-80%)
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
biopsy dx of AIH
interface hepatitis and plasma cells (don’t have to have plasma cells)
type I AIH is positive for what autoantibodies?
ANA, ASMA
type II AIH is positive for what autoantibodies?
Anti-LKM
management of AIH
- immunosuppression (prednisone, azathioprine), 2. if refractory, cyclosporine, tacrolimus, MPM, 3. liver transplant
