Autoantibodies and Autoantigens in Autoimmune Hepatitis

Christian P. Strassburg, MD, Michael P. Manns, MD

Semin Liver Dis. 2002;22(4)

Autoimmune Hepatitis Type 3

AIH type 3 is serologically defined by the presence of SLA/LP autoantibodies. However, 74% of patients also have other serological markers of autoimmunity, including SMA and AMA. AIH type 3 has a lower prevalence than AIH type 2 has, affects female patients in 90%, and has an age maximum between 20 and 40 years. This subclass of AIH is based solely on the autoantibody profile and clinically resembles AIH type 1.^[6]

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Table 1. Table 1. Definitions of Antinuclear Antibodies and Their Relation to Primary Biliary Cirrhosis
PBC-specific ANA	Less PBC-specific ANA
Anti-gp210	Anti-cyclin A
Anti-nucleoporin p62	Anti-lamin B receptor
Anti-Sp100	Anti-promyelocytic leukemia protein

Table 1. Table 1. Definitions of Antinuclear Antibodies and Their Relation to Primary Biliary Cirrhosis
PBC-specific ANA	Less PBC-specific ANA
Anti-gp210	Anti-cyclin A
Anti-nucleoporin p62	Anti-lamin B receptor
Anti-Sp100	Anti-promyelocytic leukemia protein

Table 2. Table 2. Diagnosis Score for Autoimmune Hepatitis
Parameter	Score
Gender
Female	+2
Male	0
Serum biochemistry: ratio of elevation of serum alkaline phosphatase vs.aminotransferase
> 3.0	-2
1.5-3.0	0
< 1.5	+2
Total serum globulin, -globulin or IgG: times upper normal limit
> 2.0	+3
1.5-2.0	+2
1.0-1.5	+1
< 1.0	0
Autoantibodies (titers by immunfluorescence on rodent tissues): adults
ANA, SMA or LKM-1
> 1:80	+3
1:80	+2
1:40	+1
< 1:40	0
Antimitochondrial antibody
Positive	-4
Negative	0
Hepatitis viral markers
Negative	+3
Positive	-3
Other etiological factors
History of drug usage
Yes	-4
No	+1
Alcohol (average consumption)
< 25 g/d	+2
> 60 g/d	-2
Genetic factors: HLA DR3 or DR4	+1
Other autoimmune diseases	+2
Response to therapy
Complete	+2
Relapse	+3
Liver histology
Interface hepatitis	+3
Predominant lymphoplasacytic infiltrate	+1
Rosetting of liver cells	+1
None of the above	-5
Biliary changes	-3
Other changes	-3
Seropositivity for other defined autoantibodies	+2

Table 3. Table 3. Molecular Characterization of the Major Serological Marker Autoantibodies in Autoimmune Hepatitis
Antibody	kDa	Target Antigen	Disease
Autoantigens of the endoplasmic reticulum (microsomal autoantigens)
LKM-1	50	Cytochrome P450 2D6	Autoimmune hepatitis Type 2 hepatitis C
LKM-2	50	Cytochrome P450 2C9	Ticrynafen-induced hepatitis
LKM-3	55	UGTIA	Hepatitis D-associated autoimmunity
LKM	50	Cytochrome P450 2A6	Autoimmune hepatitis Type 2 Autoimmune polyendocrine syndrome type 1 (APS-1) Hepatitis C
LM	52	Cytochrome P450 1A2	Dihydralazine-induced hepatitis Hepatitis with autoimmune polyendocrine syndrome type 1 (APS-1)
	57	Disulfidisomerase	Halothane hepatitis
	59	Carboxylesterase	Halothane hepatitis
	35	?	Autoimmune hepatitis
	59	?	Chronic hepatitis C
	64	?	Autoimmune hepatitis
	70	?	Chronic hepatitis C
Soluble autoantigens of the cytoplasm
LC1	58-62	Formiminotransferase cyclodeaminase	Autoimmune hepatitis type 2 Autoimmune hepatitis Hepatitis C?
SLA/LP	50	UGA repressor tRNA-associated protein	Autoimmune hepatitis (type 3)

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Autoantibodies and Autoantigens in Autoimmune Hepatitis

Autoimmune Hepatitis Type 3

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