Autoimmune hepatitis is a chronic inflammatory disease of the liver that results from an autoimmune attack against the liver cells. It was first described in the 1950s under various names before being termed autoimmune hepatitis in 1992. It predominantly affects females and is often diagnosed between ages 40-50. Treatment involves immunosuppressive medications like prednisone and azathioprine to induce remission of symptoms and liver inflammation. Relapses may occur upon withdrawing treatment, requiring retreatment with the same medications.
2. Since it was first described in the 1950s, this
disorder has been known by a variety of terms,
including, chronic active hepatitis, chronic
aggressive hepatitis, lupoid hepatitis, plasma
cell hepatitis, and, most commonly,
autoimmune chronic active hepatitis. In 1992,
the International Autoimmune Hepatitis Group
recommended autoimmune hepatitis as the
most appropriate term for this disease
3. EPIDEMIOLOGY & INCIDENCE
Female : male ratio of 3.6 : 1
Often diagnosed in patients in their 40s – 50s
Incidence is 0.9 – 2 per 100.000 population per
year.
5. Autoimmune hepatitis – Primary
Biliary Cirhossis overlaps
• 1-14 % of PBC patients had features of AIH
• 2 categories
AIH – PBC
overlaps
AMA + ve AIH
Autoimmune
Cholangiopathy
6. Autoimmune hepatitis – primary
sclerosing cholangitis overlaps
• Suspect when a patient with serological
evidence of AIH plus:
Pruritis
Cholestatic jaundice
Chronic ulcerative colitis
Abnormal cholangiogram
Liver biopsy showing bile duct abnormalities
No response to steroids alone
7. Clinical picture
• AIH has a heterogeneous and fluctuating
nature leading to marked variability in its
clinical manifestations.
• Some patients may present with mild to
severe nonspecific symptoms such as fatigue,
lethargy and small joint arthralgia.
8. Extrahepatic manifestations
• Hemolytic anemia
• Immune thrombocytopenia
• Type 1 diabetes
• Thyroiditis
• Ulcerative colitis
• Polyglandular autoimmune
syndrome.
Complications
• Are those seen with any
progressive liver disease.
9. Lab features
• As a general rule, aminotranferase elevations
are more striking than those of biliruben or
alkaline phosphatase.
• Hypergamma globulinemia is generally
associated with circulating autoantibodies
which is useful in the diagnosis
14. AASLD initial therapy
• Prednisone 60 mg daily or 30 mg daily plus
Azathioprine 50 mg daily
• Patients should have a minimum duration of
biochemical remission of 2 years.
• Until normal enzymes, IgG and biopsy.
• Liver transplantation should be considered in
patients with Acute liver failure.
15. AASLD recommendations regarding
relapses, treatment failure & cirrhosis
• 1st relapse after drug withdrawal should be
retreated with prednisone and AZA at the
same treatment regimen used for initial
therapy.
• Gradual withdrawal should be attempted after
at least 2 years.
• Consider using Mycophenolate mofetil or
Cyclosporin.