Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: EC:1.11.1.7 (peroxidase)
 65,474 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty five (41%) sera presented anti MPO specificity, 26 of them (74%) having a p-ANCA pattern. They were present in patients with vasculitis and isolated or predominant renal involvement, but also in 24% of Wegener patients.
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PMID:Anti-myeloperoxidase antibodies and associated diseases. 829 16

We have conducted a prospective study of 372 patients with well-defined forms of systemic vasculitis and connective tissue diseases to determine the prevalence, the antigenic specificities and the clinical associations of ANCA in such cases. These antibodies were detected by indirect immunofluorescence on ethanol-fixed neutrophils and also by enzyme-linked immunosorbent assay using myeloperoxidase (MPO) as a substrate. In our study, ANCA with a cytoplasmic immunostaining pattern were mainly found in patients with biopsy-proven Wegener's granulomatosis with or without renal involvement and pulmonary hemorrhage. Furthermore, MPO-ANCA strongly correlated with necrotizing glomerular and alveolar capillaritis, mostly in patients having a well-established diagnosis of polyarteritis nodosa.
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PMID:Anti-neutrophil cytoplasmic autoantibodies (ANCA): antigenic specificities and clinical associations. 829 18

Sera from patients with a vasculitis and controls were investigated for the presence of anti-endothelial cell antibodies(AECA), anti- neutrophil cytoplasmic antibodies(ANCA) and anti-myeloperoxidase (MPO) antibodies. Only 19% of patients with Wegener's granulomatosis and 2% of patients with microscopic polyarteritis had AECA. Our data suggests that AECA are a minor antibody system in vasculitis.
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PMID:Little evidence for anti-endothelial-cell antibodies in microscopic polyarteritis and Wegener's granulomatosis. 829 47

ANCA analysis as evaluated by concomitant determination of ANCA-IF, PR3-AB and MPO-AB in a larger cohort of dialysis patients disclosed that the prevalence of Wegener's granulomatosis and microscopic polyarteritis in Germany is 4 fold higher than hitherto believed. Low ANCA-IF titers without measurable PR3-AB or MPO-AB are not pathological- they resemble an increased autoimmune tendency of the dialysis patients. According to the european dialysis and transplantation association (EDTA)-registry the incidence of Wegener's granulomatosis (WG) and microscopic polyarteritis (MP) on haemodialysis (HD) in Germany is 0.5% and the prevalence is in the same range (1). In contrast to Germany the incidence in France and Great Britain is above 2%. This is mainly based on histologically proven diseases, which can identify only 1/3 of all affected cases, corresponding to our own experience. With the advent of ANCA serology, WG and MP can be diagnosed more easily. C-ANCA with proteinase 3-antibodies (PR3-AB) is typical of WG, and p-ANCA with myeloperoxidase-antibodies (MPO-AB) is typical of MP in our country. Since a considerable number of patients are on chronic HD without knowledge of their underlying renal disease we studied the prevalence of ANCAs in a larger cohort of HD patients.
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PMID:Prevalence of ANCAs in patients on maintenance haemodialysis. 829 48

In a 3 year period 359/3685 patients with suspected vasculitis had a positive ANCA-IF. C-ANCA were found in 146/359 and p-ANCA in 213/359 patients. P-ANCA and MPO-antibodies were more frequently associated with female gender and preglomerular vasculitis, whereas c-ANCA (WG) were more frequent in males without renal vasculitis. Detection of anti-neutrophil cytoplasmic antibodies (ANCA) has facilitated the diagnosis of Wegener's granulomatosis (WG) and microscopic polyarteritis (MP), important representatives of small vessel vasculitides (SVV). C-ANCA with proteinase 3 antibodies (PR 3-AB) are typical of WG and p-ANCA with myeloperoxidase antibodies (MPO-AB) are typical of MP in our country. We reported recently about an association between crescentic glomerulonephritis, renal vasculitis, p-ANCA, myeloperoxidase-AB and female gender in a small cohort of patients with rapidly progressive glomerulonephritis (RPGN). In the following we analyzed whether in a larger cohort of patients with SVV this preliminary statements could be confirmed.
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PMID:p-ANCA with myeloperoxidase antibodies and c-ANCA with proteinase 3 antibodies define a different vasculitis entity in patients with renal involvement. 829 53

We studied the presence of proteinase 3 (PR3), myeloperoxidase (MPO) and elastase (HLE) on the plasma membrane of neutrophils in patients with biopsy-proven Wegener's disease (WG), pANCA-positive vasculitis, control patients (SLE, rheumatoid arthritis, ankylosing spondylitis), sepsis patients and healthy donors. We found an overexpression of PR3 on the cell surface of neutrophils in WG, ANCA-associated vasculitis and during infection (sepsis). Thus PR3 becomes accessible to ANCA. Furthermore we detected intracytoplasmic IgG antibodies in PMN from patients with WG by immunoelectron microscopy and direct immunofluorescence. Our findings support the pathophysiological role of ANCA.
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PMID:Membrane surface proteinase 3 expression and intracytoplasmic immunoglobulin on neutrophils from patients with ANCA-associated vasculitides. 829 55

Sera of 108 patients with chronic inflammatory bowel disease (IBD) and 13 sera from patients with other gastrointestinal diseases were screened for antibodies against neutrophil cytoplasmic antigens (ANCA) by an indirect immunofluorescence test. 37 out of 64 sera (58%) from patients with ulcerative colitis (UC) produced a fine granular and perinuclear ANCA staining pattern ("snowdrift-like" p-ANCA) clearly different from the cytoplasmic ANCA fluorescence (c-ANCA) seen in active Wegener's granulomatosis (WG) and the typical p-ANCA pattern produced by anti-myeloperoxidase (MPO) autoantibodies. Only 1 of 44 sera from patients with Crohn's disease (CD) and none of the control sera showed positive "snowdrift-like" p- ANCA reactions. 31 out of the 37 p-ANCA positive sera (84%) were obtained from patients with high disease activity with and without longterm high dose steroids. p-ANCA titers became negative after longterm steroid therapy and following complete colectomy.
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PMID:p-ANCA of undefined specificity in ulcerative colitis: correlation to disease activity and therapy. 829 65

Anti-proteinase 3 antibodies are a subgroup of anti-neutrophil cytoplasmic antibodies (ANCA), and we have established an ELISA for their detection using high performance liquid chromatography (HPLC)-purified protein. This assay is sensitive and specific: inhibition studies have shown that despite the homology between proteinase 3 and elastase there is no cross-reactivity between the corresponding antibodies for their targets. Anti-proteinase 3 antibodies were associated most often with cytoplasmic fluorescence (17/22, 77%), but occasionally with a perinuclear (3/22, 14%) or atypical pattern (1/2). These antibodies were found in 23 out of 76 sera (30%) that were positive in an ELISA based on a crude neutrophil cytoplasmic extract, and they were associated with both 29 and 55 kD bands on Western blots. Anti-proteinase 3 antibodies were found in most individuals with active Wegener's granulomatosis (10/13, 77%), but less often in individuals with microscopic polyarteritis (2/10, 20%) or segmental necrotizing glomerulonephritis (3/6, 50%). However, anti-proteinase 3 antibodies were not detected in any of 32 sera from individuals with rheumatoid arthritis or systemic lupus erythematosus (SLE). Occasionally anti-proteinase 3 antibodies were associated with anti-glomerular basement membrane antibodies (1/11, 9%) or with anti-myeloperoxidase antibodies (1/11, 9%). IgM anti-proteinase 3 antibodies were uncommon (2/22 sera, 9%), and no IgA antibodies were demonstrated in any of 22 sera from patients with active systemic vasculitis. Significantly more individuals presented with anti-proteinase 3 antibodies in April-May-June, suggesting that an infective agent prevalent in Autumn might have a causative role in the associated diseases. Anti-proteinase 3 antibodies are the most common target antigen associated with Wegener's granulomatosis and cytoplasmic fluorescence.
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PMID:Anti-proteinase 3 antibodies, their characterization and disease associations. 830

We investigated the clinical features and outcome of 60 patients with antineutrophil cytoplasmic autoantibodies with cytoplasmic staining (C-ANCA)- and with (peri)nuclear staining (P-ANCA)-associated glomerulonephritis. Virtually all patients with C-ANCA had antibodies against proteinase 3, which corresponded with a clinical and/or histological diagnosis of Wegener's granulomatosis (WG). P-ANCA were associated with antibodies against myeloperoxidase. Although more often associated with renal-limited disease, a significant number of patients with P-ANCA had also symptoms of organs known to be preferentially affected with WG. The majority of patients presented with rapidly progressive glomerulonephritis which responded favorably to immunosuppressive therapy, albeit at the expense of a considerable number of fatal infections. Furthermore, a more protracted form of glomerulonephritis was recognized. A kidney biopsy scoring system appeared to be of value in predicting which patients would benefit most from treatment. C-ANCA and P-ANCA appear to identify a subset of patients with vasculitis that share common clinical features and a favorable response to immunosuppressive therapy.
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PMID:Clinical features and outcome in patients with glomerulonephritis and antineutrophil cytoplasmic autoantibodies. 832 48

To determine the spectrum of systemic diseases potentially associated with pauci-immune rapidly progressive glomerulonephritis (GN), most of which being considered as idiopathic, we have analyzed extra-renal manifestations, occurrence of extra-glomerular vasculitis and incidence and specificity of ANCAs in 40 patients selected only on histological criteria. Extra-renal symptoms were unexpectedly observed in all patients but one, and were suggestive of vasculitis in 24 of them. Extra-glomerular vasculitis was evidenced in 18 kidney biopsies and four biopsies from other organs. Among the 33 patients with suspected or established vasculitis, 13 had presumed or biopsy-proven Wegener's granulomatosis (WG), three had a macroscopic form of polyarteritis nodosa and 17 had a clinical presentation compatible with the so-called microscopic polyarteritis previously described in the british literature. An additional patient had clinical signs of WG without clinical and histological evidence of vasculitis. ANCAs were detected in 28/33 and 25/34 sera tested by IF and ELISA, respectively: 19 contained anti-myeloperoxidase (MPO) antibodies and 6 had anti-proteinase 3 (Pr3) activity. Anti-MPO and anti-Pr3 antibodies were present in all clinical subgroups but with various incidences: anti-MPO antibodies were surprisingly more often detected (6/12) than anti-Pr3 (4/12) in patients with suspected or histologically proven WG but anti-Pr3 antibodies were nonetheless 3- to 4-fold more frequent in WG than in non-WG systemic vasculitis (1/12) and necrotizing CGN without evidence of extra-renal vasculitis (1/10). These results strongly suggest that pauci-immune necrotizing CGNs belong to the broad spectrum of necrotizing vasculitides affecting glomerular capillaries. This hypothesis is also supported by the good response of patients to immunosuppressive treatments known for their efficacy in vasculitides, whereas these treatments are usually less successful in severe forms of extra-capillary GN with immune deposits.
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PMID:["Idiopathic" extracapillary glomerulonephritides without immune deposits are vascularitides: clinical and serologic analysis]. 836 53


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