Autoimmune Hepatitis

Posted by e-Medical PPT
Also Known as Active chronic hepatitis or chronic active hepatitis,Chronic aggressive hepatitis,Lupoid hepatitis,Plasma cell hepatitis and Autoimmune chronic active hepatitis

Chronic hepatitis of unknown etiology
Can progress to cirrhosis
Characteristics include:
 presence of autoimmune antibody
 evidence of hepatitis
 elevation of serum globulins

Accounts for 5.6% of liver transplants in the US
Affects women more than men (3.6:1)
If untreated approximately 40% die within 6 months
40% develop cirrhosis

CLASSIFICATION
TYPE 1
ANA or Anti-Smooth Muscle antibody positive
Titer usually > 1:100
10% will have an antibody to Soluble Liver antigens (SLA)
Other Antibodies: anti-DNA, ANCA, Anti-mitochondrial, Anti-Actin (AAA), cytoskeletal antibody, nuclear envelope proteins lamin A and C, plasma membrane sulfatides
Bimodal Age distribution (ages 10-20 and 45-70)
Female:male (3.6:1)
Associated with extrahepatic manifestations:
Autoimmune thyroiditis, graves disease, chronic UC
Less commonly with RA, pernicious anemia,  systemic sclerosis, ITP, SLE
40% present with acute onset of symptoms similar to toxic hepatitis or acute viral hepatitis

TYPE 2
Presence of anti-Liver/Kidney Microsome Antibodies or anti-Liver Cytosol antibody (ALC-1)

OVERLAP SYNDROMES
Primary Biliary Cirrhosis
Primary Sclerosing Cholangitis

5% of patients with chronic hepatitis C will have an ANA titer of >1:100
A homogeneous pattern of staining is more common in ANA positive autoimmune hepatitis compared to that of  ANA positive chronic hepatitis C...

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