AUTOIMMUNE HEPATITIS

Question 1

Overview (3)

Answer 1

• A progressive liver disease, more common in young/middle-aged women
• Associated with other autoimmune diseases e.g. pernicious anemia, thyroiditis
• Cause is unknown. Characterized by immunological abnormalities including hypergammaglobinemia with high IgG levels, circulating autoantibodies, and interface hepatitis with portal plasma cell infiltration on liver histology

Question 2

Clinical Features (6)

Answer 2

1- Onset: insidious with nausea, anorexia, malaise, and fatigue
2- 25% present as acute hepatitis, with rapidly progressive liver disease
3- Signs of chronic liver are often present with:
- Palmer erythema
- Spider nevi
- Hepatosplenomegaly
- Jaundice
4- Features of other autoimmune diseases are often present
5- Type 1 AIH: Usually young women. ABs: antinuclear, smooth muscle, p-ANCA
6- Type 2 AIH: Usually children. ABs: soluble liver antigen, liver/kidney microsomal antibodies (LKM)

Question 3

Investigations (4)

Answer 3

1- Circulating autoantibodies (present in most patients):
- ANA
- Anti-smooth muscle antibodies
- Soluble liver Ag antibody
- Liver/kidney microsomal antibodies
2- Hypergammaglobenimea (particularly IgG)
3- Elevated serum bilirubin and aminotransferases
4- Liver histology showing non-specific changes of chronic hepatitis, with interface hepatitis and often cirrhosis

Question 4

Indications for treatment

Answer 4

Hypergammaglobulinemia >= 2x and AST >= 5x upper limit of normal, or AST >= 10x and symptoms, or bridging necrosis (thus biopsy is necessary for guiding treatment)

Question 5

Treatment (4)

Answer 5

1- Prednisolone 30mg daily for 2-3 weeks. A subsequent reduction in the dose depends on response, but a maintenance dose of 10-15 mg is usually required.
2- Azathioprine is added as steroid-sparing agent and usually continued lifelong.
3- Combination therapy with prednisolone and azathioprine is mainstay of Tx
4- Mycophenolate, Ciclosporin, and Tacrolimus if treatment fails

Question 6

Prognosis (2)

Answer 6

1- Steroids and azathioprine therapy induce remission in over 80% of cases
2- Liver transplant may be necessary if treatment fails although the disease can recur