Autoimmune Hepatitis

Question 1

What is Autoimmune hepatitis………?

Answer 1

It is chronic hepatic inflammatory process manifested by

elevated serum aminotransaminase concentrations,

liver-associated serum autoantibodies, and/or

hypergammaglobulinemia

Question 2

The serological autoantibody profile in AIH type 1…..?

Answer 2

Anti-nuclear antibodies (ANA) and/or

Anti–smooth muscle antibody (SMA)

Question 3

The serological autoantibody profile in AIH type 2 …..?

Answer 3

AIH type 2, with positivity for anti–liver kidney microsomal type 1 antibody (anti-LKM-1)

Question 4

The targets of the inflammatory process in AIH …..?

Answer 4

Hepatocytes and to a lesser extent bile duct epithelium.

Question 5

Etiology for AIH…..

Answer 5

Autoimmune Regulator Gene - Autoimmune hepatitis arises in a genetically predisposed host after an unknown trigger leads to a T cell–mediated immune response targeting liver autoantigens.

Question 6

Main autoantigen in type 2 autoimmune hepatitis……?

Answer 6

Cytochrome P450 2D6 is the main autoantigen in type 2 autoimmune hepatitis.

Question 7

What is AIRE…….?

Answer 7

Autoimmune regulator gene (AIRE)

AIRE mutations also cause autoimmune polyendocrinopathycandidiasis-ectodermal dystrophy (also called autoimmune polyendocrinopathy syndrome), in which autoimmune hepatitis occurs in approximately 20% of patients

Question 8

Biopsy findings in AIH……?

Answer 8

Inflammatory infiltrates, consisting of lymphocytes and plasma cells that expand portal areas and often penetrate the lobule (interface hepatitis)

moderate to severe piecemeal necrosis of hepatocytes extending outward from the limiting plate

bridging necrosis

Question 9

Extrahepatic manifestations of AIH ……

Answer 9

Arthritis , vasculitis, nephritis, thyroiditis, Coombs-positive anemia, and rash.

Some patients’ initial clinical features reflect cirrhosis (ascites, hypersplenism, bleeding esophageal varices, or hepatic encephalopathy

Question 10

Lab findings in AIH ……?

Answer 10

Hypergammaglobulinemia. Serum immunoglobulin G levels usually exceed 16 g/L

Question 11

Characteristics of AIH……..?

Answer 11

female gender,

primary elevation in transaminases and not alkaline phosphatase (or GGT), elevated γ-globulin levels,

the presence of autoantibodies (most commonly antinuclear, smooth muscle, or liver-kidney microsome), and

characteristic histologic findings

Question 12

Drug of choice for AIH………?

Answer 12

Prednisone, with or without azathioprine

Question 13

What is the goal of corticosteroids treatment in AIH……?

Answer 13

The goal is to suppress or eliminate hepatic inflammation with minimal side effects

Question 14

Dosage and duration of prednisone in AIH…….?

Answer 14

Prednisone at an initial dose of 1-2 mg/kg/24 hr is continued until aminotransferase values return to less than twice the upper limit of normal.

The dose should then be lowered in 5-mg decrements over 2-4 mo until a maintenance dose of 0.1-0.3 mg/kg/24 hr is achieved.

Question 15

What is the side effects of Azathioprine….?

Answer 15

Bone marrow suppression

Question 16

The role of cholerectic agent in AIH ……?

Answer 16

The choleretic agent, ursodeoxycholic acid, is often used in biliary tract disease,

Used AIH with overlap syndrome

Question 17

PROGNOSIS of AIH …..?

Answer 17

The initial response to therapy in autoimmune hepatitis is generally prompt, with a >75% rate of remission

Transaminases and bilirubin fall to near-normal levels, often in the first 1-3 months.

When present, abnormalities in serum albumin and prothrombin time respond over a longer period (3-9 mo)

Question 18

Prognosis of AIH …….?

Answer 18

50%, relapse occurs after a variable period.

Relapse usually responds to retreatment

Liver transplantation has been successful in patients with end-stage or fulminant liver disease

Disease recurs after transplantation in approximately 30% of patients