Autoimmune hepatitis

Clinical information

Autoimmune hepatitis (AIH; previously called lupoid hepatitis, chronic active hepatitis) predominantly affects women (75% of cases). The disease manifests by an increase in bilirubin, liver enzymes and immunoglobulins, by characteristic histological changes (liver biopsy shows necrosis of the parenchyma cells with lymphocyte and plasma cell infiltration) and the presence of various autoantibodies. The disease can occur from early childhood up to old age, but is most frequent in young to middle adulthood. In Western Europe the incidence of AIH is 1.9 cases per 100,000 inhabitants per year. Untreated, AIH soon develops into liver cirrhosis. However, with low-dose immunosuppressive therapy administered in good time and consistently right up until death, patients have a normal life expectancy. In the differential diagnosis, an infection with hepatitis viruses must be ruled out through the investigation of the appropriate serological parameters.

Diagnostics

Circulating autoantibodies have come to play a significant role in the diagnosis of AIH. They occur in the majority of patients, although their role in pathogenesis is debatable. There is no clear correlation between the disease activity or prognosis and the antibody titer.

The following autoantibodies are associated with AIH: antibodies against cell nuclei (ANA), native DNA, smooth muscle (ASMA, most important target antigen: F-actin), soluble liver antigen/ liver-pancreas antigen (SLA/LP), liver-kidney microsomes (LKM-1, target antigen: cytochrome P450 IID6) and liver cytosolic antigen type 1 (LC-1, target antigen: formiminotransferase cyclodeaminase). The autoantibodies against SLA/LP that can today be detected by various EUROIMMUN enzyme immunoassays have the highest diagnostic accuracy of all antibodies involved in AIH. Anti-SLA/LP antibodies occur in AIH either alone or together with other autoantibodies. Their prevalence is only between 10 and 30%, but the predictive value is almost 100%. Essentially, every positive finding is evidence of autoimmune hepatitis (as long as the corresponding clinical symptoms are present).

Furthermore, high concentrations of autoantibodies against smooth muscles (ASMA) indicate AIH. One part of the antibodies is directed against conformational epitopes of F-actin, which are only present in frozen tissue sections or tissue cells and cannot therefore be detected by ELISA or Westernblot. In contrast to other ASMA, antibodies against F-actin are a very specific marker for type 1 AIH. With the cell line VSM47 (vascular smooth muscle) the microfilamentous (MF) fluorescence pattern can be easily and clearly differentiated from non-MF patterns, thus facilitating the diagnosis of type 1 AIH.

Selected Products

Method
Parameter
Substrate
Species/ Antigen
IIFT
liver-specific
antigens
liver
monkey
EUROLINE
positive control serum: IgG, human, 100x concentrated
for DL 1300-X G
EUROASSAY
AMA M2, LKM-1, SLA/LP
EUROASSAY strip
with antigens
IIFT
Liver Mosaic 1
liver-kidney microsomes (LKM), ANA
mitochondria (AMA), LKM
2 BIOCHIPs per field:
liver
kidney

rat
rat
IIFT
Liver Mosaic 2
liver antigens, cell nuclei (ANA)
AMA M7, heart antigens
cell nuclei (ANA)
LKM, ANA
mitochondria (AMA), LKM
smooth muscles (ASMA)
6 BIOCHIPs per field:
liver
heart
HEp-2 cells
liver
kidney
stomach

monkey
monkey
human
rat
rat
rat
EUROLINE
Liver Profile 2
(AMA M2, M2-3E, LKM-1, LC-1, SLA/LP separately)
EUROLINE
EUROASSAY
LKM-1, LC-1, SLA/LP
EUROASSAY strip
with antigens
IIFT
Liver Sreen 1
mitochondria (AMA), LKM
soluble liver antigen/
liver-pancreas antigen (SLA/LP)
LKM, ANA
smooth muscles (ASMA)
4 BIOCHIPs per field:
kidney
transfected cells

liver
stomach

rat
EU 90

rat
rat
IIFT
Liver Mosaic 3
liver antigens, cell nuclei (ANA)
skeletal muscle
cell nuclei (ANA)
LKM, cell nuclei (ANA)
mitochondria (AMA), LKM
smooth muscles (ASMA)
6 BIOCHIPs per field:
liver
musculus iliopsoas
HEp-2 cells
liver
kidney
stomach

monkey
monkey
human
rat
rat
rat
EUROLINE
Liver Profile
(AMA M2, LKM-1, LC-1, SLA/LP separately)
EUROLINE
EUROASSAY
Liver Profile
AMA M2, LKM-1, LC-1, SLA/LP
EUROASSAY strip
with antigens
EUROLINE
Autoimmune Liver Diseases
(AMA M2, M2-3E, Sp100, PML, gp210,
LKM-1, LC-1, SLA/LP, Ro-52 separately)
EUROLINE
EUROLINE
Autoimmune Liver Diseases 14 Ag
(AMA-M2, M2-3E, Sp100, PML, gp210,
LKM-1, LC-1, SLA/LP, SS-A, Ro-52, Scl-70,
CENP A, CENP B, PGDH separately)
EUROLINE
IIFT
Autoimmune liver diseases Screen 8
EUROPattern
liver antigens, cell nuclei (ANA)
F-actin
cell nuclei (ANA), EUROPattern
LKM, ANA
mitochondria (AMA), LKM
smooth muscles (ASMA)
6 BIOCHIPs per field:
liver
VSM47
HEp-2 cells
liver
kidney
stomach

monkey
rat
human
rat
rat
rat
IIFT
Liver Mosaic 8
liver antigens, cell nuclei (ANA)
F-actin
cell nuclei (ANA)
LKM, ANA
mitochondria (AMA), LKM
smooth muscles (ASMA)
6 BIOCHIPs per field:
liver
VSM47
HEp-2 cells
liver
kidney
stomach

monkey
rat
human
rat
rat
rat
IIFT
Autoimmune liver diseases Screen 9
EUROPattern
mitochondria (AMA), LKM
LKM, ANA
smooth muscles (ASMA)
F-actin
4 BIOCHIPs per field:

kidney
liver
stomach
VSM47


rat
rat
rat
rat
IIFT
Liver Mosaic 9
mitochondria (AMA), LKM
LKM, ANA
smooth muscles (ASMA)
F-actin
4 BIOCHIPs per field:
kidney
liver
stomach
VSM47

rat
rat
rat
rat
IIFT
antibodies against soluble liver antigen/
liver-pancreas antigen (SLA/LP ab control)
ELISA
soluble liver antigen/
liver-pancreas antigen (SLA/LP)
antigen-coated
microplate wells
recombinant SLA/LP
expression in E. coli
EUROASSAY
SLA/LP
EUROASSAY strip
with antigens
IIFT
soluble liver antigen/
liver-pancreas antigen (SLA/LP)
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
ELISA
cytosolic
liver antigen type 1
(LC-1)
antigen-coated
microplate wells
recombinant formiminotransferase
cyclodeaminase, expression with
baculovirus vector in insect cells
IIFT
antibodies against liver-kidney microsomes
(LKM ab control)
ELISA
liver-kidney microsomes
(LKM-1)
antigen-coated
microplate wells
recombinant cytochrome
P450 IID6
IIFT
antibodies against F-actin
IIFT
F-actin
VSM47
rat
IIFT
F-actin EUROPattern
VSM47
rat
IIFT
antibodies against smooth muscles
(ASMA control)
IIFT
smooth muscles
(ASMA)
stomach
rat
IIFT
smooth muscles (ASMA)
F-actin
stomach
VSM47
(2 BIOCHIPs per field)
rat
rat
IIFT
smooth muscles
(ASMA)
stomach
mouse
IIFT
Mosaic Basic Profile 1 EUROPattern
cell nuclei (ANA), EUROPattern
mitochondria (AMA)
smooth muscles (ASMA)
3 BIOCHIPs per field:
HEp-2 cells
kidney
stomach

human
rat
rat
IIFT
Mosaic Basic Profile 1
cell nuclei (ANA)
mitochondria (AMA)
smooth muscles (ASMA)
3 BIOCHIPs per field:
HEp-2 cells
kidney
stomach

human
rat
rat
IIFT
Mosaic Basic Profile 2 EUROPattern
cell nuclei (ANA), LKM
mitochondria (AMA), LKM
smooth muscles (ASMA)
3 BIOCHIPs per field:
liver
kidney
stomach

rat
rat
rat
IIFT
Mosaic Basic Profile 2
cell nuclei (ANA), LKM
mitochondria (AMA), LKM
smooth muscles (ASMA)
3 BIOCHIPs per field:
liver
kidney
stomach

rat
rat
rat
IIFT
Mosaic Basic Profile 25
cell nuclei (ANA), LKM
cell nuclei (ANA)
mitochondria (AMA), LKM
smooth muscles (ASMA)
4 BIOCHIPs per field:
liver
liver
kidney
stomach

rat
monkey
rat
rat
IIFT
Mosaic Basic Profile 3 EUROPattern
cell nuclei (ANA), EUROPattern
cell nuclei (ANA)
mitochondria (AMA)
smooth muscles (ASMA)
4 BIOCHIPs per field:
HEp-2 cells
liver
kidney
stomach

human
monkey
rat
rat
IIFT
Mosaic Basic Profile 3
cell nuclei (ANA)
cell nuclei (ANA)
mitochondria (AMA)
smooth muscles (ASMA)
4 BIOCHIPs per field:
HEp-2 cells
liver
kidney
stomach

human
monkey
rat
rat
IIFT
Mosaic Basic Profile 1A
cell nuclei (ANA)
mitochondria (AMA)
smooth muscles (ASMA)
3 BIOCHIPs per field:
HEp-20-10 cells
kidney
stomach

human
rat
rat
IIFT
Mosaic Basic Profile 23A
cell nuclei (ANA)
cell nuclei (ANA)
mitochondria (AMA)
smooth muscles (ASMA)
4 BIOCHIPs per field:
HEp-20-10 cells
liver
kidney
stomach

human
monkey
mouse
mouse
IIFT
Mosaic Basic Profile 3A EUROPattern
cell nuclei (ANA) EUROPattern
cell nuclei (ANA)
mitochondria (AMA)
smooth muscles (ASMA)
4 BIOCHIPs per field:
HEp-20-10 cells
liver
kidney
stomach

human
monkey
rat
rat
IIFT
Mosaic Basic Profile 3A
cell nuclei (ANA)
cell nuclei (ANA)
mitochondria (AMA)
smooth muscles (ASMA)
4 BIOCHIPs per field:
HEp-20-10 cells
liver
kidney
stomach

human
monkey
rat
rat
IIFT
Mosaic Basic Profile 3C EUROPattern
cell nuclei (ANA), EUROPattern
cell nuclei (ANA), LKM
mitochondria (AMA), LKM
smooth muscles (ASMA)
4 BIOCHIPs per field:
HEp-20-10 cells
liver
kidney
stomach

human
rat
rat
rat
IIFT
Mosaic Basic Profile 3C
cell nuclei (ANA)
cell nuclei (ANA), LKM
mitochondria (AMA), LKM
smooth muscles (ASMA)
4 BIOCHIPs per field:
HEp-20-10 cells
liver
kidney
stomach

human
rat
rat
rat
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