Clinical information
Primary sclerosing cholangitis (PSC) is a chronic cholestatic liver disease with progressive destruction of the bile ducts. The global prevalence of PSC is around 14 cases per 100,000 people. Men are more often affected than women (60 % to 80 %). The disease is mainly diagnosed between the ages of 30 and 60. PSC is observed to be more frequent in patients with chronic inflammatory bowel diseases, particularly ulcerative colitis (85% to 90%) and Crohn's disease (10 % to 15 %). For this reason, PSC patients often have autoantibodies against granulocytes (pANCA) in their serum, with the main target antigen being DNA-bound lactoferrin.
Diagnostics
Choliangography is the most important method for a reliable diagnosis of PSC. Although PSC is associated with many autoantibodies, it is not considered to be a typical autoimmune disease. However, in childhood, it may be associated with autoimmune phenomena, including high-titer autoantibodies (e.g. ANA, ANCA and ASMA). In suspected cases of overlap syndrome with autoimmune hepatitis or primary biliary cholangitis (up to 10% of cases), serological antibody testing may provide additional diagnostic information.
Methods
| Technology | Parameter | Substrate | Species | Calibration | Ig Class | Format | Order No. |
|---|---|---|---|---|---|---|---|
| EUROLINE |
EUROLINE Anti-MPO, -PR3 and -GBM (IgG) myeloperoxidase proteinase 3 (PR3) glomerular basement membrane |
EUROLINE | IgG IgG |
16 x 01 64 x 01 |
DL 1200-1601-3 G DL 1200-6401-3 G |
||
| ChLIA | IDS ANCA/GBM Control Set | 3 x 1.5 ml Control 1/2 | human serum |
2 concentrations |
IS-AI17301 | ||
| 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 |
antibodies against granulocyte cytoplasm, cytoplasmic pattern (cANCA control, PR3 ab control) |
IgG |
05 x 02 |
CA 1200-0502 | |||
| IIFT |
antibodies against granulocyte cytoplasm, perinuclear pattern (pANCA control) |
IgG |
05 x 02 |
CA 1210-0502 | |||
| IIFT |
antibodies against granulocyte cytoplasm, perinuclear pattern (pANCA control, MPO ab control) |
IgG |
05 x 02 |
CA 1211-0502 | |||
| IIFT |
cytoplasm of granulocytes (cANCA, pANCA), nuclei of granulocytes (GS-ANA) |
granulocytes, ethanol-fixed |
human | IgG IgG IgG IgG |
10 x 05 10 x 10 180 x 10 20 x 05 10 x 05 10 x 10 20 x 05 |
FA 1200-1005 FA 1200-1010 FA 1200-18010 FA 1200-2005 FB 1200-1005 FB 1200-1010 FB 1200-2005 |
|
| IIFT |
granulocytes (cANCA, pANCA) |
granulocytes, formaldehyde-fixed |
human | IgG IgG |
10 x 05 10 x 10 10 x 05 10 x 10 |
FA 1201-1005 FA 1201-1010 FB 1201-1005 FB 1201-1010 |
|
| IIFT |
Granulocyte Mosaic 13 cANCA, pANCA, GS-ANA cell nuclei (ANA), cANCA, pANCA cANCA, pANCA |
3 BIOCHIPs per field: granulocytes (EOH) HEp-2+granulocytes (EOH) granulocytes (HCHO) |
human human human |
IgG IgG IgG IgG IgG IgG |
10 x 05 10 x 10 10 x 50 120 x 10 20 x 05 20 x 10 10 x 05 10 x 10 10 x 50 20 x 05 20 x 10 |
FA 1201-1005-13 FA 1201-1010-13 FA 1201-1050-13 FA 1201-12010-13 FA 1201-2005-13 FA 1201-2010-13 FB 1201-1005-13 FB 1201-1010-13 FB 1201-1050-13 FB 1201-2005-13 FB 1201-2010-13 |
|
| IIFT |
Granulocyte Mosaic 15 cANCA, pANCA, GS-ANA cell nuclei (ANA), cANCA, pANCA |
2 BIOCHIPs per field: granulocytes (EOH) HEp-2+granulocytes (EOH) |
human human |
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 1201-1005-15 FA 1201-1010-15 FA 1201-2005-15 FA 1201-2010-15 FB 1201-1005-15 FB 1201-1010-15 FB 1201-2005-15 FB 1201-2010-15 |
|
| IIFT |
Granulocyte Mosaic 2 cANCA, pANCA, GS-ANA cANCA, pANCA |
2 BIOCHIPs per field: granulocytes (EOH) granulocytes (HCHO) |
human human |
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 1201-1005-2 FA 1201-1010-2 FA 1201-2005-2 FA 1201-2010-2 FB 1201-1005-2 FB 1201-1010-2 FB 1201-2005-2 FB 1201-2010-2 |
|
| IIFT |
EUROPLUS Granulocyte Mosaic 22 cANCA, pANCA, GS-ANA cANCA, pANCA pANCA: myeloperoxidase (MPO) cANCA: proteinase 3 (PR3) |
4 BIOCHIPs per field: granulocytes (EOH) granulocytes (HCHO) MPO BIOCHIPs PR3 BIOCHIPs |
human human |
IgG IgG IgG |
10 x 05 10 x 10 20 x 05 10 x 05 10 x 10 |
FA 1201-1005-22 FA 1201-1010-22 FA 1201-2005-22 FB 1201-1005-22 FB 1201-1010-22 |
|
| IIFT |
EUROPLUS Granulocyte Mosaic 25 cANCA, pANCA, GS-ANA cell nuclei (ANA), cANCA, pANCA cANCA, pANCA glomerular basement membrane (GBM) pANCA: myeloperoxidase (MPO) cANCA: proteinase 3 (PR3) |
6 BIOCHIPs per field: granulocytes (EOH) HEp-2+granulocytes (EOH) granulocytes (HCHO) GBM BIOCHIPs MPO BIOCHIPs PR3 BIOCHIPs |
human human human |
IgG IgG IgG IgG |
10 x 05 10 x 10 20 x 05 20 x 10 10 x 05 20 x 05 20 x 10 |
FA 1201-1005-25 FA 1201-1010-25 FA 1201-2005-25 FA 1201-2010-25 FB 1201-1005-25 FB 1201-2005-25 FB 1201-2010-25 |
|
| IIFT |
EUROPLUS Granulocyte Mosaic 32 cANCA, pANCA, GS-ANA cANCA, pANCA cell nuclei (ANA), cANCA, pANCA pANCA: myeloperoxidase (MPO) cANCA: proteinase 3 (PR3) |
5 BIOCHIPs per field: granulocytes (EOH) granulocytes (HCHO) HEp-2+granulocytes (EOH) MPO BIOCHIPs PR3 BIOCHIPs |
human human human |
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 1201-1005-32 FA 1201-1010-32 FA 1201-2005-32 FA 1201-2010-32 FB 1201-1005-32 FB 1201-1010-32 FB 1201-2005-32 FB 1201-2010-32 |
|
| IIFT |
cytoplasm of granulocytes (cANCA, pANCA), nuclei of granulocytes (GS-ANA) EUROPattern |
granulocytes, ethanol-fixed |
human | IgG EB IgG EB IgG EB IgG EB IgG EB |
10 x 05 10 x 10 120 x 10 20 x 05 20 x 10 |
FC 1200-1005 FC 1200-1010 FC 1200-12010 FC 1200-2005 FC 1200-2010 |
|
| IIFT |
granulocytes (cANCA, pANCA) EUROPattern |
granulocytes, formaldehyde-fixed |
human | IgG EB IgG EB IgG EB |
10 x 05 10 x 10 20 x 05 |
FC 1201-1005 FC 1201-1010 FC 1201-2005 |
|
| IIFT |
Granulocyte Mosaic 13 EUROPattern cANCA, pANCA, GS-ANA, EUROPattern cell nuclei (ANA), cANCA, pANCA cANCA, pANCA, EUROPattern |
3 BIOCHIPs per field: granulocytes (EOH) HEp-2+granulocytes (EOH) granulocytes (HCHO) |
human human human |
IgG EB IgG EB IgG EB IgG EB IgG EB IgG EB IgG EB IgG EB |
10 x 05 10 x 10 120 x 10 20 x 05 20 x 10 24 x 50 10 x 05 20 x 10 |
FC 1201-1005-13 FC 1201-1010-13 FC 1201-12010-13 FC 1201-2005-13 FC 1201-2010-13 FC 1201-2450-13 FW 1201-1005-13 FW 1201-2010-13 |
|
| IIFT |
Granulocyte Mosaic 15 EUROPattern cANCA, pANCA, GS-ANA, EUROPattern cell nuclei (ANA), cANCA, pANCA |
2 BIOCHIPs per field: granulocytes (EOH) HEp-2+granulocytes (EOH) |
human human |
IgG EB IgG EB IgG EB IgG EB IgG EB |
10 x 05 10 x 10 10 x 50 20 x 10 10 x 05 |
FC 1201-1005-15 FC 1201-1010-15 FC 1201-1050-15 FC 1201-2010-15 FW 1201-1005-15 |
|
| IIFT |
Granulocyte Mosaic 2 EUROPattern cANCA, pANCA, GS-ANA, EUROPattern cANCA, pANCA, EUROPattern |
2 BIOCHIPs per field: granulocytes (EOH) granulocytes (HCHO) |
human human |
IgG EB IgG EB IgG EB IgG EB IgG EB |
10 x 05 10 x 10 120 x 10 20 x 05 20 x 10 |
FC 1201-1005-2 FC 1201-1010-2 FC 1201-12010-2 FC 1201-2005-2 FC 1201-2010-2 |
|
| IIFT |
EUROPLUS Granulocyte Mosaic 22 EUROPattern cANCA, pANCA, GS-ANA, EUROPattern cANCA, pANCA, EUROPattern pANCA: myeloperoxidase (MPO), EUROPattern cANCA: proteinase 3 (PR3), EUROPattern |
4 BIOCHIPs per field: granulocytes (EOH) granulocytes (HCHO) MPO BIOCHIPs PR3 BIOCHIPs |
human human |
IgG EB IgG EB IgG EB |
10 x 05 10 x 10 20 x 10 |
FC 1201-1005-22 FC 1201-1010-22 FC 1201-2010-22 |
|
| IIFT |
EUROPLUS Granulocyte Mosaic 25 EUROPattern cANCA, pANCA, GS-ANA, EUROPattern cell nuclei (ANA), cANCA, pANCA cANCA, pANCA, EUROPattern glom. basement membrane (GBM), EUROPattern pANCA: myeloperoxidase (MPO), EUROPattern cANCA: proteinase 3 (PR3), EUROPattern |
6 BIOCHIPs per field: granulocytes (EOH) HEp-2+granulocytes (EOH) granulocytes (HCHO) GBM BIOCHIPs MPO BIOCHIPs PR3 BIOCHIPs |
human human human |
IgG EB IgG EB IgG EB IgG EB IgG EB IgG EB |
10 x 05 10 x 10 20 x 05 20 x 10 10 x 05 20 x 10 |
FC 1201-1005-25 FC 1201-1010-25 FC 1201-2005-25 FC 1201-2010-25 FW 1201-1005-25 FW 1201-2010-25 |
|
| IIFT |
EUROPLUS Granulocyte Mosaic 32 EUROPattern cANCA, pANCA, GS-ANA, EUROPattern cANCA, pANCA, EUROPattern cell nuclei (ANA), cANCA, pANCA pANCA: myeloperoxidase (MPO), EUROPattern cANCA: proteinase 3 (PR3), EUROPattern |
5 BIOCHIPs per field: granulocytes (EOH) granulocytes (HCHO) HEp-2+granulocytes (EOH) MPO BIOCHIPs PR3 BIOCHIPs |
human human human |
IgG EB IgG EB IgG EB IgG EB IgG EB IgG EB IgG EB |
10 x 05 10 x 10 120 x 10 20 x 05 20 x 10 10 x 05 10 x 10 |
FC 1201-1005-32 FC 1201-1010-32 FC 1201-12010-32 FC 1201-2005-32 FC 1201-2010-32 FW 1201-1005-32 FW 1201-1010-32 |
|
| ELISA |
ANCA-Profile ELISA (IgG) (proteinase 3, MPO, elastase, cathepsin G, BPI, lactoferrin separately) |
antigen-coated microplate wells |
native, human neutrophil granulocytes recombinant, human c-DNA expressed in human cell line |
semi-quantitative | IgG |
12 x 08 |
EA 1200-1208-1 G |
| ELISA |
Anti-PR3-hn-hr ELISA (IgG) cANCA: proteinase 3 |
antigen-coated microplate wells |
hn: native, human neutrophil granulocytes hr: recombinant, human c-DNA expressed in human cell line |
2/20/200 RU/ml | IgG |
96 x 01 |
EA 1201-9601-2 G |
| ELISA |
Anti-MPO ELISA (IgG) pANCA: myeloperoxidase |
antigen-coated microplate wells |
native, human neutrophil granulocytes |
2/20/200 RU/ml | IgG |
96 x 01 |
EA 1211-9601 G |
| EUROLINE |
positive control serum: IgG, human, 100x concentrated for DL 1200-X G |
IgG |
05 x 07 |
CL 1200-0507-2 G | |||
| EUROLINE |
EUROLINE Anti-MPO, -PR3 (IgG) myeloperoxidase proteinase 3 |
EUROLINE | IgG IgG |
16 x 01 64 x 01 |
DL 1200-1601-2 G DL 1200-6401-2 G |
||
| ChLIA | IDS MPO | antigenic coated magnetic particles | MPO |
quantitative |
50 x 01 |
IS-AI17011 | |
| ChLIA | IDS PR3-II | antigenic coated magnetic particles | PR3 |
quantitative |
50 x 01 |
IS-AI1702II1 | |
| ChLIA | Anti-PR3 ChLIA (IgG) | Antigen-coated magnetic particles | quantitative | IgG |
100 x 1 |
LA 1201-10010 G | |
| ChLIA | Anti-Myeloperoxidase ChLIA (IgG) | Antigen-coated magnetic particles | quantitative | IgG |
100 x 1 |
LA 1211-10010 G | |
| ChLIA | Control set Anti-PR3 ChLIA (IgG) | 2 x 0.4 ml control 1/2 | IgG |
02 x 02 |
LR 1201-20210 G | ||
| ChLIA | Control set Anti-Myeloperoxidase ChLIA (IgG) | 2 x 0.4 ml control 1/2 | IgG |
02 x 02 |
LR 1211-20210 G | ||
|
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