Vasculitis

Clinical information

ANCA often occur in autoimmune vasculitides of the small blood vessels – especially in granulomatosis with polyangiitis (GPA, Wegener’s granulomatosis), microscopic polyangiitis (MPA) and eosinophilic granulomatosis with polyangiitis (EGPA, Churg-Strauss syndrome) which are collectively referred to as ANCA-associated vasculitides (AAV) according to the classification system of the Chapel Hill Consensus Conference. The most important target antigens of the vasculitis-associated ANCA are proteinase 3 (PR3) and myeloperoxidase (MPO). Antibodies against PR3 are most frequently found in patients with GPA (up to 90%), while MPO ANCA have been found in MPA (40% – 80%) and EGPA (20% – 70%). Another form of vasculitis of the small blood vessels is anti-GBM disease (Goodpasture syndrome), which affects the glomerular and, in some cases, also the pulmonary capillaries. Characteristic autoantibodies are directed against the glomerular basement membrane (GBM). Up to 35% of anti-GBM positive samples are also ANCA positive. Due to clinical overlap of AAV and anti-GBM disease, it is recommended to investigate both anti-GBM and ANCA in patients with corresponding kidney damage.

Diagnostics

AAV diagnostics relies predominantly on the monospecific detection of antibodies against proteinase 3 (PR3) and myeloperoxidase (MPO), as well as the detection of ANCA using IIFT.

According to the international consensus of 2017, the use of high-quality monospecific assays is particularly recommended for the detection of PR3 and MPO in the context of GPA, MPA and EGPA diagnostics.

With the innovative Anti-PR3-hn-hr ELISA, consisting of a mixture of human native (hn) and human recombinant (hr) PR3, as well as the Anti-MPO ELISA with highly purified human MPO, EUROIMMUN offers high-quality test systems for the detection of AAV. Especially the Anti-PR3-hn-hr ELISA is characterised by a very good specificity (99%) and a significantly higher sensitivity (94%) compared to ELISAs using native antigens (88% and 78%, respectively).

EUROIMMUN offers BIOCHIPs consisting of ethanol (EOH)- and formaldehyde (HCHO)-fixed human granulocytes, which represent the standard for IIFT diagnostics. Further EUROIMMUN-exclusive BIOCHIPs, e.g. HEp-2 cells with sedimented granulocytes, further increase the diagnostic certainty. The EUROPLUS technique allows the combination of conventional cell culture substrates with defined single antigens (PR3, MPO, GBM) on one test field. This considerably simplifies the interpretation of the immunofluorescence patterns.

By means of IIFT, two ANCA types can be displayed: One is the cytoplasmic type (cANCA), which is associated with GPA and is almost always directed against PR3, and the other is the perinuclear type (pANCA), which indicates a spectrum of different diseases. The main target antigen of pANCA is mainly MPO in MPA and EGPA.

In addition, there are atypical ANCA (also x-ANCA or DNA-ANCA), which are particularly associated with chronic inflammatory bowel diseases (CIBD) and liver diseases (primary biliary cholangitis (PBC) or primary sclerosing cholangitis (PSC)). Prominent representatives of these ANCA include those directed against DNA-linked lactoferrin, cathepsin G, lysozyme or BPI.

Positive IIFT results should always be interpreted in combination with a monospecific anti-PR3 and anti-MPO test (e.g. ChLIA or ELISA). Since not all cANCA and pANCA are positive in the monospecific test, only a combination of IIFT and ChLIA/ELISA achieves high specificity and sensitivity for ANCA detection.

EUROIMMUN offers ELISA, EUROLINE, ChLIA and IIFT test systems for the detection of ANCA and antibodies against PR3, MPO and GBM.

Selected Products

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
myeloperoxidase (MPO)
proteinase 3 (PR3)
EUROLINE
EUROLINE
positive control serum: IgG, human, 100x concentrated
for DL 1200-X G
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
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
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
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)
ChLIA
Control set Anti-GBM ChLIA (IgG)
2 x 0.4 ml control 1/2
ChLIA
IDS GBM

antigenic coated magnetic particles
ChLIA
Anti-GBM ChLIA (IgG)
Antigen-coated magnetic particles
ELISA
glomerular basement membrane
(GBM)
antigen-coated
microplate wells
ELISA
sCD163
determination in urine
antibody-coated
microplate wells
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