Pathology of the GI Tract- Liver and Gallbladder (5)

Question 1

Autoimmune hepatitis has a strong association with HLA alleles. What are the 3?

Answer 1

DR3 in Caucasians; DR4 in Japanese; and DRB1 in South Americans

Question 2

In patients with AIH, what is the lymphocytic infiltrate like?

Answer 2

it is composed predominantly of CD4+ helper T cells with with CD8+ cytotoxic T cells at the interface

Question 3

the diagnosis of AIH is based on the combination of what 4 features?

Answer 3

1. exclusion of other etiologies 2. autoantibodies 3. elevation of serum IgG 4. supportive histologic findings on liver biopsy

Question 4

What are the histologic features of autoimmune hepatitis?

Answer 4

a focus of lobular hepatitis with prominent plasma cells

Question 5

What are the different types of AIH?

Answer 5

type 1 (adults) and type 2 (children)

Question 6

how is type 1 AIH characterized?

Answer 6

by the presence of either ANA or anti-smooth muscle actin (SMA) antibodies

Question 7

how is type 2 AIH characterized?

Answer 7

anti-liver kidney microsome-1 (LKM-1) antibodies

Question 8

what is the treatment of choice for AIH?

Answer 8

immunosuppression with prednisone with or without azathioprine

Question 9

what is the common presentation of AIH?

Answer 9

nonspecific symptoms such as fatigue, anorexia, nausea, and abdominal pain

Question 10

What are the 2 autoimmune cholangiopathies?

Answer 10

primary biliary cholangitis and primary sclerosing cholangitis

Question 11

what is the median age of diagnosis for PBC?

Answer 11

50 years

Question 12

what is the median age of diagnosis for PSC?

Answer 12

30 years

Question 13

what gender is most likely to be affected by PBC?

Answer 13

females

Question 14

what gender is most likely to be affects by PSC?

Answer 14

males

Question 15

what are the conditions commonly associated with PBC?

Answer 15

other autoimmune diseases Sjogren syndrome, thyroid disease, and scleroderma

Question 16

what are the conditions commonly associated with PSC?

Answer 16

IBD–> UC

Question 17

what is the serology like in PBC?

Answer 17

95% AMA-positive; 40-50% ANA positive

Question 18

what is the serology like in PSC?

Answer 18

65% ANCA positive; ANA variable; AMA typically negative

Question 19

what are the radiologic findings in PBC?

Answer 19

normal findings

Question 20

what are the radiologic findings in PSC?

Answer 20

strictures and beading of large bile ducts’ pruning of smaller ducts

Question 21

what are the duct lesions like in PBC?

Answer 21

florid duct lesions; loss of small ducts

Question 22

what are the duct lesions like in PSC?

Answer 22

there is inflammatory destruction of extrahepatic and large intrahepatic ducts

Question 23

how is PBC characterized/ defined?

Answer 23

inflammatory destruction of small- and medium-sized intrahepatic bile ducts

Question 24

what are the common clinical manifestations seen in PBC?

Answer 24

fatigue with pruritus, splenomegaly and jaundice, skin hyperpigmentation, xanthelasmas, steatorrhea, and vitamin D malabsorption (leads to osteomalacia or osteoporosis)

Question 25

what is the most characteristic finding in PBC?

Answer 25

AMA directed against PDC-E2

Question 26

how can you make the diagnosis of PBC?

Answer 26

the diagnosis can be established if two of the following are present: elevated alk phos for more than 6 months, positive test for AMA, and characteristic histologic findings

Question 27

how do you treat PBC?

Answer 27

oral ursodeoxycholic acid, a naturally occurring bile acid, slows disease progression; liver transplantation

Question 28

how is PSC characterized/defined?

Answer 28

by inflammation and obliterative fibrosis of extrahepatic and large intrahepatic ducts and dilation of preserved segments

Question 29

what is the histologic features associated with PSC?

Answer 29

a degenerating bile duct is entrapped in a dense “onion skin” concentric scar

Question 30

what are the most common presenting symptoms in PSC?

Answer 30

fatigue, pruritus, jaundice; ascending cholangitis

Question 31

what is the gold standard for diagnosis of PSC?

Answer 31

the characteristic beading seen in the large intrahepatic and extrahepatic biliary tree by ERCP/MRCP