Primary sclerosing cholangitis

Clinical information

Primary sclerosing cholangitis (PSC) is a chronic choleastic liver disease with progressive destruction of the bile ducts. PSC has a prevalence of approximately 8 to 14 patients in 100,000 people. Men are more frequently affected by the disease than women (60-80%) and the disease is mainly diagnosed at the age of 30 to 60. PSC occurs more often in patients with chronic inflammatory bowel diseases (85 to 90% ulcerative colitis and 10 to 15%  Crohn’s disease). Most patients with PSC exhibit autoantibodies against granulocytes (pANCA) in the serological test; the main target antigen is DNA-bound lactoferrin.

Diagnostics

Cholangiography is the most important method to achieve a reliable diagnosis. Although a number of autoantibodies occur in PSC, it is not a typical autoimmune disease. However, it may be associated with autoimmune phenomena in childhood, including high titers of 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 tests may provide additional diagnostic information.

Files

Autoimmune liver diseases

Differentiated ANCA determination using IIFT Mosaics

Techniques

Method
Parameter
Substrate
Species
IIFT
cytoplasm of granulocytes (cANCA,
pANCA), nuclei of granulocytes (GS-ANA)
granulocytes,
ethanol-fixed
human
IIFT
cytoplasm of granulocytes (cANCA,
pANCA), nuclei of granulocytes (GS-ANA)
EUROPattern
granulocytes,
ethanol-fixed
human
IIFT
antibodies against granulocyte cytoplasm,
cytoplasmic pattern (cANCA control, PR3 ab control)
ELISA
ANCA Profile (proteinase 3, MPO, elastase,
cathepsin G, BPI, lactoferrin separately)
antigen-coated
microplate wells
EUROLINE
positive control serum: IgG, human, 100x concentrated
for DL 1200-X G
EUROLINE
myeloperoxidase (MPO)
proteinase 3 (PR3)
EUROLINE
EUROLINE
myeloperoxidase (MPO)
proteinase 3 (PR3)
glomerular basement membrane (GBM)
EUROLINE
IIFT
granulocytes
(cANCA, pANCA)
granulocytes,
formaldehyde-fixed
human
IIFT
granulocytes
(cANCA, pANCA)
EUROPattern
granulocytes,
formaldehyde-fixed
human
ChLIA
Anti-Proteinase 3 (PR3) ChLIA (IgG)
Antigen-coated magnetic particles
ChLIA
Control set Anti-Proteinase 3 (PR3) ChLIA (IgG)
2 x 0.4 ml control 1/2
ChLIA
IDS ANCA/GBM Control Set
3 x 1.5mL Control 1/2
ChLIA
IDS PR3-II


antigenic coated magnetic particles
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
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
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
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
IIFT
Granulocyte Mosaic 2
cANCA, pANCA, GS-ANA
cANCA, pANCA
2 BIOCHIPs per field:
granulocytes (EOH)
granulocytes (HCHO)

human
human
IIFT
Granulocyte Mosaic 2 EUROPattern
cANCA, pANCA, GS-ANA, EUROPattern
cANCA, pANCA, EUROPattern
2 BIOCHIPs per field:
granulocytes (EOH)
granulocytes (HCHO)

human
human
ELISA
cANCA:
proteinase 3
(PR3-hn-hr)
antigen-coated
microplate wells
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
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
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
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
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
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
IIFT
antibodies against granulocyte cytoplasm,
perinuclear pattern (pANCA control)
ChLIA
IDS MPO

antigenic coated magnetic particles
ELISA
pANCA:
myeloperoxidase (MPO)
antigen-coated
microplate wells
IIFT
antibodies against granulocyte cytoplasm, perinuclear pattern
(pANCA control, MPO ab control)
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