Autoimmune hepatitis (AH) is an immune mediated inflammatory liver disease with a prevalence of 31.2 cases per hundred thousand persons in the US and a female predominant, occurring three times as often in women as in men.
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.
The peak age at presentation is between 40 and 60 years.
Its prevalence is slightly higher in white population than Hispanic, Asian or Black populations.
It is commonly associated with other autoimmune diseases.
Crohn’s disease is 7.2 times more likely among patients with autoimmune hepatitis as those among without autoimmune hepatitis.
The association of autoimmune hepatitis and psoriasis indicates that there is 3.5 times as much disease among patients with the disease.
More than 80% have hypergammaglobulinemia.
The pathogenesis is in completely understood, but triggers: a toxin, infection, or drug drives accuracy immunologic targeting of hepatocytes in a host with a genetic predisposition to the disease.
Normal liver immune intolerance is lost due to impairment of regulatory T cell function, activation of adaptive immune cells by autoantigens, and production of pro-inflammatory cytokines that promote hepatocyte injury.
AH is diagnosed in the basis of serologic and histologic features rather than on symptoms.
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.
Besides elevation of liver function, tests, AST, auto immune hepatitis is typically associated with antibodies to nuclear antigens, smooth muscle antigens, liver-kidney microsomal type one, soluble liver, antigens, and liver-pancreas antigens.
Liver biopsy, reveals interface hepatitis, characterized by inflammation, extending from the portal tract into the portal zone with necrosis of peripheral hepatitis and destruction of the limiting plate, and plasma cel infiltration of the portal tracts.
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.
Early diagnosis and treatment is critical to preventing complications, which include: cirrhosis, hepatocellular, carcinoma, G.I. bleeding, liver failure, and need for liver transplantation, and death.
Fulminant presentation may also occur.
Treatment:
Treatment of a age involves the use of immunosuppressive agents to prevent disease progression.
Therapy is steroids, frequently in combination with aztathioprine.
The combination of a budenoside and azathioprine is more effective than that of prednisone plus azathioprine with higher incidence to biochemical remission at six months (60% versus 39%)) and less frequent steroid side effects.
In patients 789with cirrhosis budesonide should not be used due to impaired first pass metabolism.
In 10-20% of patients steroid therapy results are inadequate and significant side effects occur in 5-10% of patients.