18-5: Autoimmune Hepatitis and Cholangiopathies

Question 1

What are the HLA associations in autoimmune hepatitis

Answer 1

Caucasians DR3
Japanese DR4
South America DRB1

Question 2

What are the four features on which the diagnosis of autoimmune hepatitis is based?

Answer 2

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

Question 3

which subtype of autoimmune hepatitis is associated with adults and children

Answer 3

Type 1 adults

Type 2 children

Question 4

what is the predominant treatment for autoimmune hepatitis

Answer 4

prednisone w,w/o azathioprine

Liver transplantation may be necessary for cirrhotic pts

Question 5

acute presentation of autoimmune hepatitis can be confused for what other pathologies

Answer 5

acute viral or drug induce hepatitis

more commonly sx are non-specific fatigue, anorexia, nausea, vomiting

Question 6

between PBC and PSC which primarily affects men

Answer 6

primary sclerosing cholangitis

Question 7

what is primary biliary cholangitis

Answer 7

autoimmune disease characterized by inflammatory destruction of small and medium sized intrahepatic bile ducts

Question 8

What is the etiology of PBC

Answer 8

t lymphocyte mediated attack on on small interlobular bile ducts.

Question 9

describe clinical features of PBC

Answer 9

• Asymptomatic - elevated serum alk phos
• Symptomatic - pruritus and fatigue
• splenomegaly and jaundice in advance disease
• hypercholesterolemia
• antimitochondrial antibodies

Question 10

how do you establish dx of PBC

Answer 10

2 or more of the following

elevated alk phos; >6 mo
positive antimitochondrial antibodies
histologic findings

Question 11

what kind of cancer is PBC associated with

Answer 11

HCC

Question 12

describe PSC

Answer 12

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

Male predominance

Question 13

describe the clinical features of PSC

Answer 13

• 1/2 of pts are asymptomatic at presentation; found during screening UC; elevated alk phos
• fatigue, pruritus and jaundice
• ascending cholangitis can be initial presentation

Question 14

what is the gold standard for PSC diagnosis

Answer 14

beading seen in large intrahepatic and extrahepatic biliary tree by endoscopic/magnetic retrograde cholangiopancreaticography