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SYNDROME
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DISTINGUISHING
FEATURES
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Overlap syndromes
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Autoimmune hepatitis
and primary biliary cirrhosis
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Mitochondrial antibodies
Histologic cholangitis
Hepatic copper deposition
Cholestatic laboratory changes
Responsiveness to corticosteroid therapy
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Autoimmune hepatitis and
primary sclerosing cholangitis
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Chronic ulcerative colitis
Histologic cholangitis
Cholestatic laboratory changes
No responsiveness to
corticosteroid therapy
Abnormal cholangiogram
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Autoimmuine hepatitis
and
chronic viral infection
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Autoimmune-predominant
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Titer of smooth-muscle or antinuclear antibodies >1:320
Titer of smooth-muscle and antinuclear antibodies >1:40
Piecemeal necrosis (interface hepatitis), lobular hepatitis,
and portal plasma cell infiltrates
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Viral-predominant
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Titer of smooth-muscle or antinuclear antibodies <1:320
Antibodies to liver/kidney microsome type 1 and hepatitis C viremia
Portal lymphoid aggregates, steatosis, or bile duct injury
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Outlier syndromes
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Autoimmune cholangitis
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Absence of mitchondrial
antibodies
Antinuclear antibodies
Smooth-muscle antibodies common
Histologic features of bile duct injury
Concurrent cholestatic laboratory changes
Antibodies to carbonic anhydrase
Normal cholangiogram
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Cryptogenic chronic hepatitis
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Absence of smooth-muscle, antinuclear, or liver/kidney microsome
type 1 antibodies at presentation
Histologic findings identical to those of autoimmune hepatitis
HLA-B8, HLA-DR3, or HLA-A1-B8-DR3 common
Possible antibodies to soluble liver antigen or liver-pancreas
Possible late appearance of conventional autoantibodies
Responsiveness to corticosteroid therapy
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