ANCA Profile ELISA
产品名称：ANCA Profile ELISA
产 地：Demeditec
产品货号：DE7040
产品规格：96 Tests
产品说明：
Special remarks:
The acronym ANCA (Antineutrophil Cytoplasmic Autoantibodies) is defined by an accumulation of autoantibodies with specificity against different granulocytic, monozytic and probably endothelial cytoplasmic antigens.
Autoantibodies against endocellular components of neutrophile granulocyte (PMN) are known for a longer period. At first, in 1964 they have been described as granulocyte specific factor directed against anti nuclear components (GS-ANA). Under the term ANCA, they were first reported in 1982 in a few patients who had necrotizing glomerulonephritis without immune deposits, but the relevance was still unclear. The next report of ANCA was in 1984 were these autoantibodies has been observed in four patients with systemic vasculitis and three of whom had also necrotizing glomerulonephritis. In 1985, a collaborative study in the Netherlands and Denmark described these factors, showing a high sensitivity for active Wegener's granulomatosis, as APCA (Anti Cytoplasmic Antibodies) and they suggested that APCA could be used as a marker for disease activity. In 1988 further investigations substantiated the association of ANCA with Wegener's Granulomatosis and polyarteritis nodosa, and also noted that ANCA occur in patients with pauciimmune ("idiopathic") necrotizing and cresenticglomerulonephritis who have no evidence for extrarenal disease. Proteinase 3 (PR3), showing a central accentuated pattern (cANCA) has been described as major antigen for Wegener's Granulomatosis. A new pattern, showing a high specificity for the microscopic Polyangiitis, with perinuclear fluorescence (pANCA) was also observed in 1988. At first, only Myeloperoxidase (MPO) was described as antigen. In the last years, newer investigations discovered and characterized a couple of new pANCA antigens:
Elastase, Cathepsin G, Lysozyme and Lactoferrin.
In the meantime, PR3 and MPO are well defined as reliable serological markers for a definite group of primary systemic vasculitides (PSV), which were also named ANCA associated vasculitides (AAV). The occurrence of AAV is clearly higher than supposed. The incidence is 1.5 per 1000 and in the group of older persons nearly 5 per 1000. The clinical appearance of the AAV is characterized trough manifestations in lung, kidney and respiratory tract.
Up to now, ANCA screening has been done with immunofluorescence techniques, but often there have been difficulties in the evaluation and in clinical findings. Therefore, the results have to be scrutinized with counter examinations on other cells or in other test systems like ELISA. Moreover it was not possible to differentiate the single cANCA and pANCA antigens.
Proteinase 3:
The major antigen for the cANCA reactivity is the neutral serin protease 3 (synonyms: p29, AGP7, Wegener autoantigen), which belongs to the Trypsin/Chymotrypsin family. In 1988 several groups showed that the antigen is a protein with a molecular weight of 29 kDa. PR3 was already described in 1973 by Ohlsson and Olsson under the name neutrophile collagenase. In the meantime it seems certain, that autoantibodies against PR3 are highly specific as serological marker for the diagnosis of Wegener's granulomatosis (specificity: initial phase 50%, generalization phase > 90%).
Moreover there is a correlation between the concentration of the autoantibodies and the disease activity.
Myeloperoxidase:
Myeloperoxidase was first isolated by Agner in 1941, who gave it the name "verdoperoxidase", because of its green color and ability to catalyze peroxidase reactions. It is MPO that gives pus its greenish tone. In nearly 60% of the pANCA findings MPO is the major antigen. The occurrence of autoantibodies against MPO is classified as relevant marker for the rapid progressive nephritis.
Moreover these antibodies occur in 70-90% in all patients with serious kidney injury. Over and about they have also been detected at the Churg-Strauß-Syndrom (CSS), Microscopic Polyangiitis (MPA) and other vasculitis diseases. The concentration of the autoantibodies correlates well with the disease activity of MPA. MPA is also characterized by clinical manifestations of lung, kidney and respiratory tract, but these manifestations are, in contrast to WG, not granulomatous.
However, these antibodies have, in contrast to the high specificity of PR3 antibodies for WG, a minor specificity of 60% in the diagnosis of MPA. The absence of autoantibodies against MPO and PR3, by simultaneous detection of ANA can be used as a tool for differential diagnosis between AAV and SLE induced vasculitis.
BPI:
Bactericidal permeability-increasing protein, BPI is a membrane-located protein of 55kDa molecular weight and is classed as an ANCA-antigen of polymorph-nuclear granulocytes and monocytes that bind endotoxin. Its autoantibodies are now classified as cANCA. Due to BPIs high affinity to lipopolysaccharides its anti-microbial effect against Gram-negative bacteria is significant. BPI is splitted and thus inactivated by using elastase or other serine protease. Autoantibodies against BPI are above all detected in chronically infectious intestinal diseases such as Morbus Crohn or colitis ulcerosa. In contrast to anti-MPO and anti-PR3 autoantibodies, those against BPI seem not to have any association with vasculitis.
Elastase:
Elastase is a serine protease with a sequence homology of 54% to that of proteinase 3. It occurs mainly in polymorph-nuclear neutrophilic granulocytes (PMN), in macrophages and endothelial cells. The dismantling of proteoglycans by neutrophiles is mainly due to elastase' proteolytic activity. Furthermore, elastase participates decisively in tissue destruction connected with emphysemas and rheumatoid arthritis. Autoantibodies against this antigen are generally associated with inflammatory rheumatic disorders, e.g. rheumatoid arthritis and vasculitis.
Cathepsin G:
The cathepsins belong to a group of intracellular proteases mainly found in lysosomes, especially of the spleen, the liver and the kidney. Cathepsin G is a serine protease and a further pANCA antigen. It participates to a great part in the destruction of osteid tissue as of its hydrolytic properties. The autoantibodies against Cathepsin G occur mainly in collagenosis and other related inflammatory rheumatic diseases, e.g. SLE, Sjögren syndrome and Felty syndrome.
Lysozyme:
In 1922, Lysozyme (LZ) was accidental discovered by Alexander Flemming. LZ is a glykosidase consisting of 129 amino acid residues with a molecular weight of 14,6 kDa, which decomposes the glycosidic bond between C-1 of MNAc and C-4 of GlcNAc. Lysozyme is localized in the azurophilic as well as in the specific granules of neutrophiles and in extracellular liquid compartments like tears and salivary, where it spreads out his antimicrobial activities against invading bacteria. LZ belongs also to the pANCA and autoantibodies against Lysozyme occur in higher frequency in rheumatoid vasculitis and inflammatory bowel disease like colitis ulcerosa.
Lactoferrin:
Lactoferrin (LF) is an iron-binding protein, with a molecular weight of 77-93 kDa, which occurs in high concentrations in secretions at mucosa surfaces, in tears and in milk. LF also resides in the specific granules of polymorphnuclear neutrophil leukocytes (PMN) and becomes exocytosed upon PMN activation. During active inflammatory disease, raised serum levels of LF can be measured.
The physiological anti microbial effect of Lactoferrin depends on its iron-binding capacity, because most of the bacteria require iron for their own physiological pathways. LF inhibits myelopoiesis, prevents complement activation and prevents the formation of hydroxl radicals. It is quite possible that LF has several important roles, like secretory IgA, as a non-specific antiphlogistic defense factor at mucosal surfaces. LF belongs to the pANCA, depending on the redistribuiton from the granules toward the nuclei, upon ethanol fixation. Autoantibodies against Lactoferrin occur in higher frequency in patients with rheumatoid vasculitis (RV), colitis ulcerosa (CU) and primary sclerosing cholangitis (PSC).
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