Characterized by chronic liver inflammation, autoantibodies and increased serum levels of gamma globulin.
Has no pathognomonic feature.
A chronic inflammatory disease of the liver resulting from loss of immunologic tolerance to hepatic tissue.
Unknown cause.
Occurs primarily in young-to-middle age females with a 4:1 female: male ratio.
More than 80% have hypergammaglobulinemia.
Liver biopsy essential for diagnosis and determining the severity of the disease.
Liver function transaminase abnormalities not predictive of histologic injury or extent of fibrosis.
Liver biopsy helpful in excluding other liver diseases.
Biopsy shows typical interface hepatitis consisting of lymphoplasmacytoid inflammatory infiltrate.
Plasma cells are classically present but may be few or absent in liver biopsy specimens in 34% of cases.
Traditionally associated with antinuclear antibodies (ANA), anti-smooth muscle antibodies (ASMA) and antibodies the liver-kidney microsomes (anti-LKM) and these tests should be part of work up in patients with suspected disease.
Positive ANA present in two thirds of patients.
Autoimmune hepatitis may present with fatigue, lethargy, anorexia, nausea, abdominal pain, itching, and arthralgia of small joints.
Diarrhea is not common with this process.
Associated with eosinophilia.
Elevated ferritin level may occur.
Common among first-degree relatives of children with autoimmune hepatitis.
Typical homogeneous or speckled immunofluorescence pattern of ANA analysis.
Diagnosis based on 4 components: serum IgG level, the presence and level of autoantobodies, absence of viral markers, and histologic features on liver biopsy.
The diagnosis of Wilson’s disease should be ruled out before diagnosis of autoimmune hepatitis is made.
Accounts for 11-23% cases of chronic liver disease.
Onset ofen insidious with fatigue and hyperbilirubinemia.
Fulminant presentation may also occur.
Therapy is steroids, frequently in combination with aztathioprine.
In 10-20% of patients steroid therapy results are inadequate and significant side effects occur in 5-10% of patients.