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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 enzyme and immunoglobulin levels and by characteristic histological changes seen on liver biopsy: necrosis of the parenchyma cells with lymphocyte and plasma cell infiltration. Various autoantibodies occur as well. 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 timely low-dose immunosuppressive therapy given consistently for the rest of the patient’s life, life expectancy is normal. Differential diagnostics should include hepatitis virus infection, ruled out by testing for 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 the pathogenesis is still unclear. There is also 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 muscles (ASMA, most important target antigen: F-actin), SLA/LP, liver-kidney microsomes (LKM-1, target antigen: cytochrome P450 2D6) and liver cytosolic antigen type 1 (LC-1, target antigen: formiminotransferase cyclodeaminase). Of all of these antibodies, autoantibodies against SLA/LP, which can today be detected by various EUROIMMUN enzyme immunoassays, have the highest specificity for 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 %. Furthermore, high concentrations of ASMA indicate AIH. A large part of the antibodies is directed against conformational epitopes of F-actin, which are best preserved in frozen tissue sections or tissue cells. In contrast to other ASMA, antibodies against F-actin are a very specific marker for type I AIH. The VSM47 (vascular smooth muscle) cell line allows fibrillar fluorescence patterns to be easily and reliably distinguished from non-fibrillar patterns, facilitating the diagnosis of type 1 AIH by indirect immunofluorescence. In addition, the determination on triple sections of rat tissue (liver, kidney, stomach; LKS) can be used for diagnosis. The multiparametric line blots – EUROLINE Autoimmune Liver Diseases profiles – enable efficient confirmation of all relevant autoantibodies.

Methods
Technology Parameter Substrate Species Calibration Ig Class Format Order No.
IIFT cell nuclei (ANA) HEp-2 cells
(1 BIOCHIP per field)
human IgG
IgG
IgG
IgG
10 x 50
120 x 10
20 x 05
20 x 10
FA 1520-1050-R
FA 1520-12010-R
FA 1520-2005-R
FA 1520-2010-R
IIFT cell nuclei (ANA) HEp-20-10 cells
(1 BIOCHIP per field)
human IgG
IgG
IgG
IgG
10 x 50
120 x 10
20 x 05
20 x 10
FA 1522-1050-R
FA 1522-12010-R
FA 1522-2005-R
FA 1522-2010-R
IIFT antibodies against liver-kidney microsomes
(LKM ab control)
IgG 05 x 02
CA 1320-0502
IIFT antibodies against F-actin IgG 05 x 02
CA 1651-0502
IIFT antibodies against smooth muscles
(ASMA control)
IgG 05 x 02
CA 1710-0502
IIFT soluble liver antigen/
liver-pancreas antigen (SLA/LP)
transfected cells
control transfection
(2 BIOCHIPs per field)
EU 90
EU 90
IgG
IgG
10 x 05
10 x 10
FA 1302-1005-50
FA 1302-1010-50
IIFT F-actin VSM47 rat IgG
IgG
10 x 05
10 x 10
FA 1651-1005
FA 1651-1010
IIFT smooth muscles
(ASMA)
stomach rat IgG
IgG
IgG
IgG
10 x 05
10 x 10
20 x 05
20 x 10
10 x 05
10 x 10
20 x 05
20 x 10
FA 1710-1005
FA 1710-1010
FA 1710-2005
FA 1710-2010
FB 1710-1005
FB 1710-1010
FB 1710-2005
FB 1710-2010
IIFT smooth muscles (ASMA)
F-actin
stomach
VSM47
(2 BIOCHIPs per field)
rat
rat
IgG
IgG
10 x 05
20 x 10
10 x 05
FA 1710-1005-1
FA 1710-2010-1
FB 1710-1005-1
IIFT F-actin EUROPattern VSM47 rat IgG PI
IgG PI
10 x 05
10 x 10
FC 1651-1005
FC 1651-1010
ELISA Anti-SLA/LP ELISA (IgG)
soluble liver antigen/ liver-pancreas antigen
antigen-coated
microplate wells
recombinant SLA/LP
expression in E. coli
2/20/200 RU/ml IgG 96 x 01
EA 1302-9601 G
ELISA Anti-LC-1 ELISA (IgG)
cytosolic liver antigen type 1
antigen-coated
microplate wells
recombinant formiminotransferase
cyclodeaminase, expression with
baculovirus vector in insect cells
semi-quantitative IgG 96 x 01
EA 1307-9601 G
ELISA Anti-LKM-1 ELISA (IgG)
liver-kidney microsomes
antigen-coated
microplate wells
recombinant cytochrome
P450 IID6
2/20/200 RU/ml IgG 96 x 01
EA 1321-9601 G
IIFT Liver Mosaic 1
liver-kidney microsomes (LKM), ANA
mitochondria (AMA), LKM
2 BIOCHIPs per field:
liver
kidney

rat
rat
IgG
IgG
IgG
10 x 05
10 x 10
20 x 05
10 x 05
10 x 10
20 x 05
FA 1300-1005-1
FA 1300-1010-1
FA 1300-2005-1
FB 1300-1005-1
FB 1300-1010-1
FB 1300-2005-1
IIFT Liver Screen 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
IgG
IgG
10 x 05
10 x 10
FA 1300-1005-21
FA 1300-1010-21
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
IgG
IgG
IgG
10 x 05
10 x 10
20 x 05
10 x 05
10 x 10
20 x 05
FA 1300-1005-8
FA 1300-1010-8
FA 1300-2005-8
FB 1300-1005-8
FB 1300-1010-8
FB 1300-2005-8
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
IgG
IgG
10 x 05
10 x 10
10 x 05
10 x 10
FA 1300-1005-9
FA 1300-1010-9
FB 1300-1005-9
FB 1300-1010-9
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
IgG PI
IgG PI
IgG PI
IgG PI
10 x 05
10 x 10
20 x 05
20 x 10
FC 1300-1005-9
FC 1300-1010-9
FC 1300-2005-9
FC 1300-2010-9
EUROLINE positive control serum: IgG, human, 100x concentrated
for DL 1300-X G
IgG 05 x 07
CL 1300-0507 G
EUROLINE EUROLINE Liver Profile 2 (IgG)
(AMA M2, M2-3E, LKM-1, LC-1, SLA/LP separately)
EUROLINE IgG
IgG
16 x 01
64 x 01
DL 1300-1601-2 G
DL 1300-6401-2 G
EUROLINE EUROLINE Liver Profile (IgG)
(AMA M2, LKM-1, LC-1, SLA/LP separately)
EUROLINE IgG
IgG
16 x 01
64 x 01
DL 1300-1601-3 G
DL 1300-6401-3 G
EUROLINE EUROLINE Autoimmune Liver Diseases (IgG)
(AMA M2, M2-3E, Sp100, PML, gp210,
LKM-1, LC-1, SLA/LP, Ro-52 separately)
EUROLINE IgG
IgG
IgG
16 x 01
50 x 01
64 x 01
DL 1300-1601-4 G
DL 1300-5001-4 G
DL 1300-6401-4 G
EUROLINE EUROLINE Autoimmune Liver Diseases 14 Ag (IgG)
(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 IgG
IgG
16 x 01
50 x 01
DL 1300-1601-5 G
DL 1300-5001-5 G
EUROLINE EUROLINE Autoimmune Liver Diseases 9 Ag plus F-Actin (IgG)
(AMA-M2, M2-3E, Sp100, PML, gp210,
LKM-1, LC-1, SLA/LP, F-Aktin und Ro-52)
EUROLINE IgG
IgG
IgG
16 x 01
50 x 01
64 x 01
DL 1300-1601-9 G
DL 1300-5001-9 G
DL 1300-6401-9 G
EUROASSAY EUROASSAY AMA-M2, LKM-1, SLA/LP (IgG) EUROASSAY strip
with antigens
IgG DA 1300-1003 G
EUROASSAY EUROASSAY Liver Profile (IgG)
AMA M2, LKM-1, LC-1, SLA/LP
EUROASSAY strip
with antigens
IgG DA 1300-1003-3 G
ChLIA IDS LKM-1 antigenic coated magnetic particles LKM-1 quantitative
50 x 01
IS-AI18021
ChLIA IDS AMA (M2)
MIT3
antigenic coated magnetic particles MIT3 quantitative
50 x 01
IS-AI18011
ChLIA IDS Liver Control Set 3 x 1.5 ml Control 1/2 human serum 2 concentrations
IS-AI18301
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
IgG
IgG
IgG
10 x 05
10 x 10
20 x 05
10 x 05
10 x 10
20 x 05
FA 1800-1005-1
FA 1800-1010-1
FA 1800-2005-1
FB 1800-1005-1
FB 1800-1010-1
FB 1800-2005-1
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
IgG
IgG
IgG
IgG
IgG
10 x 05
10 x 10
120 x 10
20 x 05
20 x 10
10 x 05
10 x 10
20 x 05
20 x 10
FA 1800-1005-2
FA 1800-1010-2
FA 1800-12010-2
FA 1800-2005-2
FA 1800-2010-2
FB 1800-1005-2
FB 1800-1010-2
FB 1800-2005-2
FB 1800-2010-2
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
IgG
IgG
IgG
10 x 05
10 x 10
20 x 05
10 x 05
10 x 10
20 x 05
FA 1800-1005-3
FA 1800-1010-3
FA 1800-2005-3
FB 1800-1005-3
FB 1800-1010-3
FB 1800-2005-3
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
IgG
IgG
IgG
IgG
10 x 05
10 x 10
20 x 05
20 x 10
10 x 05
10 x 10
20 x 05
20 x 10
FA 1802-1005-3
FA 1802-1010-3
FA 1802-2005-3
FA 1802-2010-3
FB 1802-1005-3
FB 1802-1010-3
FB 1802-2005-3
FB 1802-2010-3
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
IgG
IgG
IgG
IgG
10 x 05
10 x 10
20 x 05
20 x 10
10 x 05
10 x 10
20 x 05
FA 1812-1005-3
FA 1812-1010-3
FA 1812-2005-3
FA 1812-2010-3
FB 1812-1005-3
FB 1812-1010-3
FB 1812-2005-3
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
IgG
IgG
IgG
IgG
10 x 10
120 x 10
20 x 05
20 x 10
FC 1800-1010-2
FC 1800-12010-2
FC 1800-2005-2
FC 1800-2010-2
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
IgG PI
IgG PI
IgG PI
IgG PI
10 x 05
10 x 10
20 x 05
20 x 10
FC 1802-1005-3
FC 1802-1010-3
FC 1802-2005-3
FC 1802-2010-3
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
IgG PI
IgG PI
IgG PI
10 x 05
10 x 10
20 x 05
FC 1812-1005-3
FC 1812-1010-3
FC 1812-2005-3
* For research use only, not for in vitro diagnostic use in the sense of EU directive 98/79/EC.
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