Primary biliary cholangitis

Clinical information

Primary biliary cholangitis (PBC) is an immune-mediated chronic inflammatory cholestatic liver disease of unknown aetiology. The disease is characterised by female predominance (>90%) with most cases observed between the ages of 40 and 60. PBC incidence in different parts of the world is estimated to be 4 to 31 cases/million per year. PBC is marked by lymphocellular infiltration around the small intra-hepatic biliary ducts (bile canaliculi) and the build-up of bile (cholestasis). The disease often begins with unspecific, very varying general symptoms, such as itching (pruritus), fatigue and pain in the upper right region of the abdomen. An obstructive jaundice develops after a varying period of time. The increase in serum lipids is an important indicator for PBC. Histologically, changes occur in the liver corresponding to a chronic, non-suppurative destructive cholangitis: granulating pericholangitis, i.e. slowly progressing destruction of the small and medium-sized biliary ducts with subsequent fibrosis, the final stage of which is complete cirrhosis. In addition to the liver, often other organs with exocrine functions are also affected, above all the lachrymal and salivary glands and the pancreas.

Diagnostics

The diagnosis of PBC includes liver function tests (determination of alkaline phosphatase, aspartate transaminase and alanine transaminase), the determination of serum lipids, screening for anti-mitochondrial antibodies (AMA) and anti-nuclear antibodies (ANA) and the differentiation from other chronic inflammatory diseases of the liver, such as chronic viral hepatitis, autoimmune hepatitis or primary sclerosing cholangitis.

The detection of AMA is of great importance in the diagnosis of PBC. Antibodies against the M2 antigen are the most sensitive and specific diagnostic marker. These antibodies can be found in 94% of PBC patients. High-titer anti-M2 antibody seropositivity is an important tool in the diagnosis of PBC and a very powerful predictor of future development of PBC in patients without significant liver function disorders or symptoms suggestive of cholestatic diseases. Besides AMA, ANA may also be found in about one third of patients with PBC by indirect immunofluorescence. Promyelocytic leukaemia (PML) proteins and Sp100, which generate a nuclear dot pattern in IIFT, and two components of the nuclear pore complex (gp210 and p62) that have been specifically associated with a perinuclear pattern have been identified as specific ANA target antigens in PBC.

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 Sp100
(nuclear granula, nuclear dots; associated with PBC)
IIFT
antibodies against nuclear membrane
IIFT
mitochondria (AMA)
kidney
rat
IIFT
test performance control
(AMA, IgG)
IIFT
AMA/ASMA IIFT (KR/SR) EUROPattern
mitochondria (AMA)
smooth muscles (ASMA)
2 BIOCHIPs per field:
kidney
stomach

rat
rat
IIFT
mitochondria (AMA)
smooth muscles (ASMA)
kidney
stomach
(2 BIOCHIPs per field)
rat
rat
EUROASSAY
AMA Profile
(AMA M2, M4, M9 separately)
EUROASSAY strip
with antigens
IIFT
mitochondria (AMA)
smooth muscles (ASMA)
kidney
stomach
(2 BIOCHIPs per field)
mouse
mouse
IIFT
EUROPLUS
mitochondria (AMA)
M2 antigen
2 BIOCHIPs per field:
kidney
M2 BIOCHIPs

rat
IIFT
EUROPLUS
mitochondria (AMA)
smooth muscles (ASMA)
cell nuclei (ANA)
M2 antigen
4 BIOCHIPs per field:
kidney
stomach
HEp-2 cells
M2 BIOCHIPs

rat
rat
human
IIFT
antibodies against mitochondria
(AMA M2 control; associated with PBC)
ELISA
AMA M2-3E
antigen-coated
microplate wells
native, pyruvate dehydrogenase
complex, bovine heart plus
recombinant M2 fusion antigen (BPO)
IIFT
antibodies against mitochondria
(AMA M2 control; associated with PBC),
control serum with indication of titer
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 13B EUROPattern
cell nuclei (ANA), EUROPattern
cell nuclei (ANA), LKM
mitochondria (AMA), LKM
smooth muscles (ASMA)
4 BIOCHIPs per field:
HEp-2 cells
liver
kidney
stomach

human
rat
rat
rat
IIFT
Mosaic Basic Profile 13B
cell nuclei (ANA)
cell nuclei (ANA), LKM
mitochondria (AMA), LKM
smooth muscles (ASMA)
4 BIOCHIPs per field:
HEp-2 cells
liver
kidney
stomach

human
rat
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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