Autoimmune Hepatitis

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)

 

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

  1. Describe the typical patient with autoimmune hepatitis. Young, white female, third to fifth decade
  2. What is the gold standard in the diagnosis of autoimmune hepatitis? Liver biopsy.

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google photo

You are commenting using your Google account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s

This site uses Akismet to reduce spam. Learn how your comment data is processed.