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 approximately 4 to 31 cases per 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, widely varying general symptoms, such as itching (pruritus), fatigue and pain in the right upper abdomen. Jaundice develops after a varying period of time. An increase in serum lipids is an important indicator for PBC. Histologically, changes in the liver corresponding to a chronic, nonsuppurative destructive cholangitis can be observed: 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, other organs with exocrine functions are also often affected, primarily the lachrymal and salivary glands and the pancreas.
Diagnostics
PBC diagnostics include liver function tests (determination of alkaline phosphatase, aspartate transaminase and alanine transaminase), the determination of serum lipids, screening tests 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 for the diagnosis of PBC. Antibodies against the M2 antigen are the most sensitive and specific diagnostic marker. These antibodies can be detected 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 for early detection of the disease in patients with early-stage PBC who do not yet have 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 IFA, 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.
| 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 |
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 |
|
| IIFT |
antibodies against mitochondria (AMA M2 control; associated with PBC) |
IgG |
05 x 02 |
CA 1622-0502 | |||
| IIFT | mitochondria (AMA) | kidney | 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 1620-1005 FA 1620-1010 FA 1620-2005 FA 1620-2010 FB 1620-1005 FB 1620-1010 FB 1620-2005 FB 1620-2010 |
|
| IIFT |
mitochondria (AMA) smooth muscles (ASMA) |
kidney stomach (2 BIOCHIPs per field) |
rat rat |
IgG IgG IgG IgG IgG |
10 x 05 10 x 10 10 x 50 20 x 05 20 x 10 10 x 05 10 x 10 10 x 50 20 x 05 20 x 10 |
FA 1620-1005-1 FA 1620-1010-1 FA 1620-1050-1 FA 1620-2005-1 FA 1620-2010-1 FB 1620-1005-1 FB 1620-1010-1 FB 1620-1050-1 FB 1620-2005-1 FB 1620-2010-1 |
|
| 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 |
IgG IgG |
10 x 05 10 x 10 10 x 05 10 x 10 |
FA 1620-1005-5 FA 1620-1010-5 FB 1620-1005-5 FB 1620-1010-5 |
|
| IIFT |
AMA/ASMA IIFT (KR/SR) EUROPattern mitochondria (AMA) smooth muscles (ASMA) |
2 BIOCHIPs per field: kidney stomach |
rat rat |
IgG IgG IgG IgG |
10 x 05 10 x 10 20 x 05 20 x 10 |
FC 1620-1005-1 FC 1620-1010-1 FC 1620-2005-1 FC 1620-2010-1 |
|
| ELISA | Anti-M2-3E ELISA (IgG) |
antigen-coated microplate wells |
native, pyruvate dehydrogenase complex, bovine heart plus recombinant M2 fusion antigen (BPO) |
2/20/200 RU/ml | IgG |
96 x 01 |
EA 1622-9601 G |
| EUROASSAY |
EUROASSAY AMA Profile (IgGM) (AMA M2, M4, M9 separately) |
EUROASSAY strip with antigens |
IgGM | DA 1620-1003-1 O | |||
| EUROLINE |
EUROLINE AMA Profile (IgGM) (separate: AMA-M2, M2-3E, M4, M9) |
EUROLINE | IgGM IgGM |
16 x 01 64 x 01 |
DL 1620-1601-1 O DL 1620-6401-1 O |
||
| 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 |
IgG IgG IgG |
10 x 05 10 x 10 20 x 05 10 x 05 10 x 10 20 x 05 |
FA 1805-1005-13 FA 1805-1010-13 FA 1805-2005-13 FB 1805-1005-13 FB 1805-1010-13 FB 1805-2005-13 |
|
| 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 |
IgG PI IgG PI IgG PI |
10 x 10 20 x 05 20 x 10 |
FC 1805-1010-13 FC 1805-2005-13 FC 1805-2010-13 |
|
|
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