Chronic and autoimmune hepatitis

Question 1

List causes of chronic hepatitis

Answer 1

HBV, HCV, HDV
Autoimmune
drugs
Hepatocellualr degeneration- Wilson’s disease

Question 2

List four disease that may simulate chronic hepatitis

Answer 2

Alcoholic hepatitis
Non-alcoholic steatohepatitis
Alpha -1- Antitrypsin deficiency
Genetic Hemochromatosis

Question 3

Autoimmune hepatitis presents with increased ALT/AST, prolonged INR, circulating autoantibodies, and _________

Answer 3

hypergammaglobulinemia

Question 4

Autoimmune hepatitis usually responds to treatment with _______

Answer 4

prednisone or azathioprine

Question 5

Describe the pathogenesis of autoimmune hepatitis

Answer 5

Loss of self-tolerance, genetic predisposition
Helper T-cells infiltrate portal zones, react with hepatocyte antigens
Plasma cells frequently present in large numbers

Question 6

The autoantibodies seen in autoimmune hepatitis have _____ value but are not proven to have a _______ role

Answer 6

diagnostic role but not pathogenic

Question 7

What types of autoantibodies are seen in autoimmune hepatitis?

Answer 7

Type 1: 80% of cases
ANA: Anti nuclear Ab (ds DNA)
ASMA: Anti smooth muscle Ab (actin, troponin)

Type 2: 4% of cases
Anti-LKM1: Anti liver kidney microsomal (CYP2D6)
Anti-LC1: Anti-liver cytosol (Formiminotransferase cyclo demaminase)
Often young girls, European

Other antibodies:
Anti-SLA: Anti soluble liver antigen Ab (Cytokeratin 8,18)
Atypical p-ANCA

Question 8

Autoimmune hepatitis is commonly associated with:

Answer 8

autoimmune thyroiditis
Grave’s disease
Rheumatoid arthritis
Ulcerative colitis

Question 9

Does autoimmune hepatitis recur after liver transplant?

Answer 9

Possible

Question 10

List three causes of metabolic liver disease

Answer 10

Genetic Hemochromatosis
Wilson’s Disease
Alpha-1-Antitrypsin Deficiency

Question 11

Hereditary hemochromatosis is an autosomal ______ disorder due to mutations of HFE gene

Answer 11

recessive

Question 12

Describe the underlying pathology of genetic hemochromatosis

Answer 12

body always interprets as low iron state- ferroportin is always active due to failure of hepcidin regulation

Question 13

How is hereditary hemochromatosis diagnosed?

Answer 13

increased transferrin
increased ferritin
HFE gene analysis
liver biopsy for iron quantification

Question 14

Wilson’s disease is an autosomal ______ disorder of a _____ transporter

Answer 14

recessive

copper

Question 15

In Wilson’s disease, copper accumulates in the:

Answer 15

liver, brain (basal ganglia), cornea, kidney

Question 16

How is Wilson’s disease diagnosed?

Answer 16

Screening tests:
Ceruloplasmin: low
24 hr urine copper: high

Confirmatory tests:
Liver biopsy for copper quantification (high)

Question 17

How is Wilson’s disease treated?

Answer 17

Chelating agents: Trientene, Pencillamine

Liver Transplantation : acute hepatic failure, advanced cirrhosis

Question 18

a1 antitrypsin is a ___________ synthesized in the rER of hepatocytes

Answer 18

serine protease inhibitor

Question 19

_______ is the most common deficiency variant of a1 antitrypsin

Answer 19

PiZZ

Question 20

Contrast the pathogenesis of liver vs lung disease in a1 antitrypsin deficiency

Answer 20

Liver Disease:
Accumulation of abnormal protein in ER–> cell death, fibrosis, cirrhosis

Lung Disease:
Decreased serum A-1 AT level–> elastase destroys elastic tissue around alveoli–> premature emphysema

Question 21

Liver biopsy in a1 antitrypsin deficiency characteristically shows

Answer 21

intracellular globules

Question 22

How is a1 antitrypsin deficiency treated?

Answer 22

Liver disease: Liver transplantation- CURATIVE

Lung disease: Alpha-1-trypsin administration

Question 23

Copper is regulated at the level of the ______, iron is regulated at the level of the _____

Answer 23

liver

enterocytes

Question 24

What does a biopsy in autoimmune hepatitis show?

Answer 24

plasma cell infiltrate, interface hepatitis