–Autoimmune hepatitis is a chronic disorder characterized by continuing hepatocellular necrosis and inflammation.
-It can progress to cirrhosis and liver failure.
-Though usually a disease of young women (70% of cases occur in females), autoimmune hepatitis can occur in either sex at any age.
-most common in whites and Northern Europeans.
–The cause is unknown, but believed to be autoimmunity.
–Two subtypes of disease have been described based on the autoantibodies present: type 1 AIH—ANA and/or ASMA (anti-actin); type 2 AIH—anti-LKM (anti–liver kidney microsomal).
-Affected younger persons are often positive for HLA-B8 and -DR3; older patients are often positive for HLA-DR4
Symptoms & Signs:
Clinical Manifestations: Fever, Fatigue, anorexia, jaundice, abdominal pain, epistaxis, cirrhosis.
Extrahepatic Manifestations: Rash, erythema nodosum,pleurisy, pericarditis, anemia, arthralgias, keratoconjunctivitis sicca, xerostomia, thyroiditis, hemolytic anemia, nephritis.
Diagnosis: Diagnosis is based on many factors.
Demographics: 70% of cases in females.
History: Exclude other known causes of liver disease
Serology: presence of specific autoantibodies, which include antinuclear antibodies (ANAs), smooth-muscle antibodies (SMAs), antibodies to liver and kidney microsome type 1 (anti-LKM1), and anti-liver cytosol type 1 (anti-LCI), perinuclear antineutrophil cytoplasmic antibodies (aANCAs)
Biochemistry: Elevated aminotransferases and serum IgG, hypergammaglobulinemia
Clinical: response to immunosuppressive treatment
Histology: portal tract inflammation that extends into the parenchyma (interface hepatitis, plasma cells, rosettes,bridging necrosis)
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Treatment:
Medications: Immunosuppressive therapy with either steroids (prednisone, budesonide) or/and azathioprine
Surgery: Liver transplantation
Immunization: Vaccinate against hepatitis A and B.
Prognosis:
-Approximately 50% of patients with autoimmune hepatitis will die within 5 years without treatment. Death may result from complications of cirrhosis, hepatic failure or hepatic coma.
-The majority of patients (90%) with autoimmune hepatitis achieves complete remission within 3 months
- Describe the typical patient with autoimmune hepatitis. Young, white female, third to fifth decade
- What is the gold standard in the diagnosis of autoimmune hepatitis? Liver biopsy.