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GI/Liver > Lec 22 Autoimmune > Flashcards

Lec 22 Autoimmune Flashcards

1
Q

Who gets primary biliary cirrhosis?

A
  • women more than men

- mean age 50 yo

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2
Q

What is pathogenesis of primary biliary cirrhosis?

A
  • have external trigger [environment or infectious] and underlying susceptibility
  • causes autoimmune attack and destriuction of intralobular bile ducts
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3
Q

What diseases are associated with primary biliary cirrhosis?

A
  • celiac
  • sjogren
  • rheumatoid arthritis
  • thyroid
  • scleroderma
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4
Q

What lab values can you use to diagnose biliary cirrhosis?

A
  • high alk phos/GGTP
  • high cholesterol
  • high conjugated bilirubin
  • anti-mitochondiral antibody [AMA]
  • high serum IgM
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5
Q

What disease is associated with anti mitochondrial antibody?

A

primary biliary cirrhosis

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6
Q

What do you see on histology in primary biliary cirrhosis?

A

florid duct lesion = lymphocytic infiltration and attack of bile duct

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7
Q

What are symptoms of primary biliary cirrhosis?

A
  • pruritis
  • jaundice
  • dark urine
  • light stools
  • hepatosplenomegaly
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8
Q

What is prognosis of primary biliary cirrhosis?

A

slowly progressive

good prognosis

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9
Q

Who gets primary sclerosing cholangitis?

A
  • men

- onset 24-25 yo

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10
Q

What is pathogenesis of primary sclerosing cholangitis?

A
  • unknown cause

- may be hypersentivity; or immune mediated; or assocaited wtih infection

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11
Q

How does primary sclerosing cholangtis present?

A
  • elevation of LFTs
  • pruritis
  • cholangitis –> fever, chills, RUQ pain, jaundice
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12
Q

What labs do you see in PSC?

A
  • high alk phos/GTP
  • high conjugated bilirubin
  • high cholesterol
  • no specific antibody tests –> can be pANCA [anti neutrophil cytoplasmic antibody] OR anti-SMA [anti-smooth muscle] or ANA [anti nuclear]
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13
Q

What histo/imaging findings with primary sclerosing cholangtiis?

A
  • onion skin fibrosis around bile duct

- alternating strictures and dilations with beading of intra and extra hepatic bile ducts

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14
Q

What diseases associated with primary sclerosing cholangitis?

A

IBD [ulcerative colitis > crohns]

cholangiocarcinoma

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15
Q

What are 2 complications of primary sclerosing cholangitis?

A

secondary biliary cirrhosis
cholangiocarcinoma
colon cancer risk increased 10x

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16
Q

What is treatment for primary sclerosing cholangitis?

A
  • dilation of dominant stricture

- ursodeoxycholic acid to increase bile flow

17
Q

Who gets autoimmune hepatitis?

A

adolescent or post menopausal women

18
Q

How does autoimmune hepatitis present?

A

can be acute: RUQ pain, fever, chills, jaundice

or can be insidious with abnormal LFTs, fatigue, aches

19
Q

What are diagnostic criteria for autoimmune hepatitis?

A
  • positive antinuclear antibody [ANA] or smooth muscle antibody [SMA] or LKM1 [liver kidney muscle antibody]
  • high total IgG
  • high ALT/AST
  • liver biopsy to rule out other causes
20
Q

What is treatment of autoimmune hepatitis?

A

long term immunosuppression: prednisone

21
Q

What is prognosis of autoimmune hepatitis?

A

75% mortality if untreated; good pronosis if treated

22
Q

What is the target for autoimmune hepatitis?

A

hepatocytes

23
Q

What is genetics of wilson disease?

A
  • autosomal recessive

- mutation in chr 13 ATP7B gene responsible for excretion of copper into the bile

24
Q

What is pathogenesis of wilson disease?

A
  • have accumulation of copper in hepatocytes –> causes mitochondrial damage and lipid oxidation –> hepatocellular death and release of unbound copper into blood
25
Q

Where does copper deposit in wilsons?

A
  • brain
  • kidneys
  • cornea
26
Q

What stain to test for copper deposition in the liver?

A

rhodamine stain

27
Q

What do you see in liver in wilson disease?

A
  • elevated ALT/AST
  • chronic hepatitis
  • liver failure
  • cirrhosis
  • can lead to hepatocellular carcinoma
28
Q

What neuro signs do you see in wilsons?

A

progressive movement disorder

  • mildtremor, speech/writing problems, basal ganglia degerneration leading to parkinsonian symptoms
  • dimentia, dyskinesia
29
Q

What heme signs of wilsons?

A

hemolytic anemia

30
Q

How do you diagnose wilson disease?

A
  • kayser fleisher rings = copper deposited in cornea
  • urinary copper
  • low serum ceruloplasmin
  • liver biopsy containing copper
31
Q

What is treatment for wilson disease?

A
  • copper chelator = penicillamine
  • zinc = blocks intestinal copper absorption
  • low copper diet
32
Q

What foods should you avoid in wilsons disease?

A

mushrooms, nuts, chocolate, dried fruit, liver, shellfish

33
Q

What is genetic cause of hemochromatosis? What HLA type associated?

A
  • mutation in C282Y or H63D of HFE gene on chr 6

- associated with HLA-A3

34
Q

What is pathogenesis of hemochromatosis?

A
  • mutation in HFE gene –> impaired iron sensing and absorption
    have excess iron absorbed from GI tract
35
Q

Where does iron deposit in hemochromatosis?

A
  • liver
  • heart
  • pituiary and thyroid
  • pancreas
  • joints
  • gonad
36
Q

What is action of HFE normally?

A

liver cells sense plasma iron and absorb iron via HFE
HFE regulates synthesis of hepcidin
hepcidin inhibits iron secretion by duodenum and tells macrophages of GI to stop taking up iron

37
Q

What levl of hepcidin in hemochromatosis?

A

low hepcidin b/c of defect in HFE

38
Q

What is classic triad of hemochromatosis?

A
  • cirrhosis
  • bronze skin
  • diabetes
39
Q

What lab levels in hemochromatosis?

A
  • high fasting transferrin sat > 50%
  • ferritin > 1000
  • high irin
  • low TIBC

GI/Liver (36 decks)

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