Vasculitic syndromes and renal injury This review emphasizes our increasing understanding of renal involvements in vasculitic syndromes. (1994 Apr)
vasculitic syndromes and renal injury This review emphasizes our increasing understanding of renal involvements in vasculitic syndromes . systemic vasculitis of various size vessels ( large , medium , and small ) can be classified according to the ANCA positivity . large vessel vasculitides are usually ANCA negative and the occurrence of renal vasculitis or glomerulonephritis is rare . inflammatory aneurysm of abdominal aorta exhibits peculiar renal injury . In this disease , marked fibrosis of periaorta often induces hydronephrosis . On the other hand , ANCA is positive in most of small vessel vasculitides . C ANCA is specific for wegener s granulomatosis and P ANCA for pauci immune crescentic glomerulonephritis with or without pulmonary hemorrhage . pathogenetic role of ANCA in renal involvements in small vessel angitis can be well explained from the standpoint of ANCA cytokine sequence theory . another modern topic is lupus vasculopathy . renal vascular changes in lupus nephritis are various in relation to the presence of anti DNA antibody , anti phospholipid antibody , and anti endothelial cell antibody , or long administration of corticosteroids .

Antigen specific radioimmunoassays for anti neutrophil cytoplasmic antibodies in the diagnosis of rapidly progressive glomerulonephritis. (1991 Nov)
antigen specific radioimmunoassays for anti neutrophil cytoplasmic antibodies in the diagnosis of rapidly progressive glomerulonephritis . circulating anti neutrophil cytoplasmic antibodies ( ANCA ) have been described in most patients with pauci immune necrotizing and crescentic glomerulonephritis . A 29 kDa serine protease ( p29 or proteinase 3 ) and myeloperoxidase are the two best characterized antigens recognized by ANCA . The study presented here was conducted to define the diagnostic value of assays for antibodies against these two antigens in rapidly progressive glomerulonephritis . radioimmunoassays were developed for anti p29 and anti myeloperoxidase antibodies , with purified antigens , and the results of the radioimmunoassays were compared with those obtained by immunofluorescence tests for ANCA . We performed assays on serum samples from 123 patients with the syndrome of rapidly progressive glomerulonephritis , as well as from 200 blood bank donors and from 717 additional control patients . without knowledge of the results of ANCA tests , the renal pathologic findings in the 123 patients with rapidly progressive glomerulonephritis were analyzed , and 42 were classified as pauci immune necrotizing and crescentic glomerulonephritis , 18 were classified as anti glomerular basement membrane nephritis and 63 were classified as other forms of renal disease . We found radioimmunoassays to be more reliable in the diagnosis of pauci immune necrotizing and crescentic glomerulonephritis than immunofluorescence testing . By radioimmunoassay , ANCA were found in 40 of 42 patients ( 95 sensitivity ) with pauci immune necrotizing and crescentic glomerulonephritis ( 14 …

The increase of antiglomerular basement membrane antibody following pauci immune type crescentic glomerulonephritis. (2005 Apr)
The increase of antiglomerular basement membrane antibody following pauci immune type crescentic glomerulonephritis . A 50 year old woman was admitted because of high fever and fatigue . proteinuria , hematuria , and elevated BUN ( 47 . 8 mg / dl ) and creatinine ( 3 . 4 mg / dl ) suggested rapidly progressive glomerulonephritis . The serological study revealed all negative results for rheumatoid factor , antinuclear antibody , serum cryoglobulins , MPO ANCA , PR3 ANCA , and anti streptolysin O . antiglomerular basement membrane ( GBM ) antibody , as assessed by elisa , was 11 EU ( normal , 10 ) . kidney biopsy on the eighth hospital day demonstrated pauci immune type crescentic glomerulonephritis without ANCA . methylprednisolone pulse therapy ( 500 mg / day , 3 days ) and 45 mg / day prednisolone orally were started . At 3 weeks after kidney biopsy , the anti GBM antibody value increased from 11 EU / ml to 116 EU / ml , and MPO and PR3 ANCA were still negative . HLA type was DR8 and DR 15 ( 2 ) , with a genotype of HLA DRB1 08021 and HLA DRB1 15011 . The present case suggests that HLA DR15 plays an important role on antibody production against alpha 3 ( IV ) NC1 autoantigen after severe nephritis or tissue damage .

Anti neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis. (1988 Jul)
Anti neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis . Anti neutrophil cytoplasmic autoantibodies have been found in patients with systemic arteritis and glomerulonephritis . We studied the disease distribution and antigen specificity of these autoantibodies . Anti neutrophil cytoplasmic autoantibodies were identified by indirect immunofluorescence microscopy in 27 of 35 patients with idiopathic necrotizing and crescentic glomerulonephritis , in whom the manifestations of disease ranged from injury limited to the kidney to systemic arteritis . The incidence and titers of the autoantibodies did not differ between patients with disease limited to the kidney and those with systemic disease . Anti neutrophil immunostaining was detected in 5 of 11 patients with lupus nephritis , 4 of 71 patients with other renal diseases , and none of 50 normal controls . This distribution of autoantibodies was confirmed by an enzyme linked immunosorbent assay ( elisa ) using neutrophil lysate as antigen . according to elisa , anti neutrophil cytoplasmic autoantibodies were found to be specific for constituents of primary granules . Two types of autoantibodies were identified ; one with reactivity with myeloperoxidase on elisa produced an artifactual perinuclear immunostaining of alcohol fixed neutrophils , and another with no reactivity with myeloperoxidase on elisa produced diffuse cytoplasmic immunostaining . The presence of the same serologic marker in patients with kidney limited and arteritis associated necrotizing and crescentic glomerulonephritis , including wegener s granulomatosis and polyarteritis nodosa , suggests that these clinically …

Antineutrophil cytoplasmic autoantibodies and associated diseases : a review. (1990 Aug)
antineutrophil cytoplasmic autoantibodies and associated diseases : a review . antineutrophil cytoplasmic autoantibodies ( ANCA ) are specific for constituents of neutrophil primary granules and monocyte lysosomes . there are different types of ANCA with different specificities . By indirect immunofluorescence microscopy using alcohol fixed neutrophils as substrate , two major categories of ANCA can be recognized , one with cytoplasmic staining ( C ANCA ) and the other with artifactual perinuclear staining ( P ANCA ) . Some C ANCA have specificity for proteinase 3 ( PR3 ANCA ) and some P ANCA have specificity for myeloperoxidase ( MPO ANCA ) , but there are additional C ANCA and P ANCA specificities . ANCA are found in the blood of patients with necrotizing systemic vasculitis , such as wegener s granulomatosis and polyarteritis nodosa , and patients with idiopathic crescentic glomerulonephritis . The glomerular lesion in patients with systemic and renal limited ANCA associated diseases is the same , ie , a pauci immune necrotizing and crescentic glomerulonephritis . No matter where the vascular lesions of ANCA associated disease are ( eg , kidney , lung , gut , muscle , skin ) , they are characterized by necrotizing inflammation and a paucity of immune deposits . The distribution of disease correlates to a degree with the ANCA specificity , although there is substantial overlap . For example , patients with wegener s granulomatosis most often have C ANCA and patients with renal limited disease most often have P …

The syndrome of lung hemorrhage and nephritis is usually an ANCA associated condition. (1996 May)
The syndrome of lung hemorrhage and nephritis is usually an ANCA associated condition . background : In the absence of evidence of arteritis or wegener s granulomatosis , the syndrome of lung hemorrhage and nephritis has been commonly associated with anti glomerular basement membrane ( GBM ) antibodies . however , it has been increasingly recognized that many cases are associated with antineutrophil cytoplasmic antibodies ( ancas ) . objective : To review available clinical and pathologic findings to determine the diseases accounting for lung hemorrhage and nephritis . methods : We studied the records of 750 patients from whom serum samples were sent to our laboratory for anti GBM antibody assays between 1981 and 1993 and found 88 patients with evidence of lung hemorrhage and nephritis . serum samples were retested , using current methods , for anti GBM antibodies ( against noncollagenous 1 domain of the alpha 3 chain of type IV collagen ) and for antibodies to proteinase 3 and myeloperoxidase the two types of ANCA of diagnostic value . results : Of 88 patients with evidence of lung hemorrhage and nephritis , 48 had ancas , six had anti GBM antibodies , and seven had both . In 48 patients with ancas , the pathologic findings that accounted for the pulmonary renal syndrome were pauci immune necrotizing and crescentic glomerulonephritis and pulmonary capillaritis . Only eight had convincing evidence ( during life ) of wegener s granulomatosis and only one other had documented arteritis . In …

Neutrophil contribution to the crescentic glomerulonephritis in SCG / Kj mice. (2004 Jun)
neutrophil contribution to the crescentic glomerulonephritis in SCG / Kj mice . background : myeloperoxidase specific anti neutrophil cytoplasmic auto antibody ( MPO ANCA ) has been a useful diagnostic marker in systemic vasculitis with crescentic glomerulonephritis ( CrGN ) . It is highly suspected that the antigenic enzyme MPO released from activated neutrophils is involved in these lesions . We evaluated the relationship between neutrophil functions including peripheral neutrophil counts and renal lesions in SCG / Kj mice as a model of ANCA associated CrGN and vasculitis . methods : peripheral neutrophil counts , the plasma levels of MPO ANCA and tumour necrosis factor alpha ( TNF alpha ) were measured . The capacity of MPO release and superoxide generation were evaluated as neutrophil activity . The renal lesions were estimated by grade of proteinuria , histopathological lesion , such as glomerular neutrophil infiltration and active or chronic renal injury scores with crescent formation . results : MPO ANCA and TNF alpha levels were higher than those of normal mice c57bl / 6 even before overt proteinuria ; subsequently , peripheral neutrophils increased . In the phase of nephritis with low grade proteinuria , the spontaneous release of MPO from peripheral neutrophils increased , while superoxide generation increased before spontaneous MPO release occurred . In addition , the renal lesion in histological observations was aggravated with ageing and the glomerular neutrophil infiltration was positively correlated with MPO ANCA levels , as well as with histological indices of nephritis , …

Antineutrophil cytoplasmic autoantibodies in poststreptococcal nephritis. (1994 Mar)
antineutrophil cytoplasmic autoantibodies in poststreptococcal nephritis . Sera from 210 patients with apsgn , were tested for the presence of ANCA ( IgG isotype ) . indirect immunofluorescence ( IF ) on ethanol fixed human PMNs was used , and for those positive sera , elisa kits for PR3 ( proteinase 3 ) and MPO ( myeloperoxidase ) was performed . ANCA were detected in 9 ( 18 out of 210 cases ) in a predominantly diffuse cytoplasmic staining pattern in 14 cases ( 77 ) , and in a perinuclear pattern in the remaining 4 cases ( 22 ) . Anti MPO was found in 4 cases ( C ANCA 3 ; P ANCA 1 ) and anti PR3 was always negative . The presence of ANCA was significantly associated with a more severe glomerular disease as assessed by the serum creatinine value and the crescents formation . longitudinal studies performed in 11 cases have shown that raised levels of these autoantibodies may persist for at least six months , without relationship with disease activity . further studies are required to dilucidate the specificity of these autoantibodies , and if its presence is either an epiphenomenon of the heterogeneous humoral immune response in streptococcal infection , or they play some pathogenic role in apsgn .

Antigens of antineutrophil cytoplasmic autoantibodies in recognizing a novel neutrophil granule antigen in lupus nephritis and its association with photosensitivity … (2002 Dec)
antigens of antineutrophil cytoplasmic autoantibodies in recognizing a novel neutrophil granule antigen in lupus nephritis and its association with photosensitivity and oral ulceration objective : To investigate whether there are some unknown target antigens of antineutrophil cytoplasmic autoantibodies ( ANCA ) in patients with lupus nephritis ( LN ) and the prevalence and the clinical significance of these unknown target antigens . methods : serum was collected from 72 renal biopsy proven lupus nephritis ( LN ) patients . mixed neutrophil granules were separated from normal human peripheral neutrophils and soluble acid extracts in non reducing conditions were used as antigens in western blot analysis to detect ANCA in the serum sample of LN patients . results : serum from seme LN patients could blot a few bands . interestingly , serum from 14 / 72 ( 19 . 4 ) of the patients recognized a novel 69 , 000 protein band and that from 10 / 72 ( 13 . 9 ) sera recognized a 55 , 000 protein band that might be the bactericidal / permeability increasing protein ( BPI ) . The 69 , 000 target antigen was different from the known target ANCA antigens such as cathepsin G and lactoferrin . further study revealed that the percentages of patients with photosensitivity and oral ulcer in anti 69 , 000 autoantibody positive group were significantly higher than those in the anti 69 , 000 autoantibody negative group ( 57 . 1 vs 12 . 1 , P …

Cytoplasmic antineutrophil cytoplasmic antibody positive pauci immune glomerulonephritis associated with infectious endocarditis. (2006 Dec)
cytoplasmic antineutrophil cytoplasmic antibody positive pauci immune glomerulonephritis associated with infectious endocarditis . renal deterioration often occurs in cases of infectious endocarditis ( IE ) , but , IE associated nephritis with rapidly progressive glomerulonephritis ( RPGN ) is rare . patients with severe infection ( e . g . , IE ) sometimes show positivity for cytoplasmic antineutrophil cytoplasmic antibodies ( C ANCA ) . therefore , diagnosis and treatment are very difficult in cases of RPGN with IE and positivity for C ANCA . Such cases are rare , only 12 have been reported in the english literature . herein , we describe the case of a 50 year old man who presented with RPGN with IE and tested positively for C ANCA . He was referred to our hospital because of leg edema , purpura and renal dysfunction . laboratory tests revealed serum creatinine elevation and positivity for C ANCA and proteinase 3 specific ( PR3 ) ANCA . RPGN and acute renal failure were diagnosed . hemodialysis and steroid therapy were started . streptococcus oralis was isolated by blood culture . transthoracic echocardiography revealed grade III mitral valve insufficiency with two vegetations . therefore , IE was diagnosed . The steroid therapy was stopped , and antibiotic therapy was begun . because there was no improvement , surgical therapy was performed . The operation was successful , but the patient died of brain hemorrhage . Our experience in this case indicates C / PR3 ANCA positive …

Pulmonary renal syndromes. (2007 Jun)
pulmonary renal syndromes . pulmonary renal syndromes or lung kidney syndromes are clinical syndromes defined by a combination of diffuse alveolar haemorrhage ( DAH ) and glomerulonephritis . pulmonary renal syndromes are not a single entity , but are caused by a wide variety of diseases , including various forms of primary systemic vasculitis ( especially wegener s granulomatosis and microscopic polyangiitis ) , goodpasture s syndrome ( associated with autoantibodies to the alveolar and glomerular basement membrane ) and systemic lupus erythematosus . The diagnosis rests on the identification of particular patterns of clinical , radiologic , pathologic and laboratory features . serologic testing is important in the diagnostic work up of patients presenting with a pulmonary renal syndrome . The majority of cases of pulmonary renal syndrome are associated with ancas , either c ANCA or p ANCA , due to autoantibodies against the target antigens proteinase 3 and myeloperoxidase respectively . The antigen target in goodpasture s syndrome is type IV collagen , the major component of basement membranes . diffuse alveolar haemorrhage is characterized by the presence of a haemorrhagic bronchoalveolar lavage ( BAL ) in serial BAL samples . In the clinical setting of an acute nephritis syndrome , percutaneous renal biopsy is commonly performed for histopathology and immunofluorescence studies . treatment of generalized ANCA associated vasculitis consists of corticosteroids and immunosuppressive agents such as cyclophosphamide ( as induction therapy ) or azathioprine ( as maintenance therapy once remission has been achieved ) . The …

Anti neutrophil cytoplasmic antibody MPO ANCA related with disease activity of vasculitis myeloperoxidase ( MPO ) specific anti neutrophil cytoplasmic … (2006 May)
Anti neutrophil cytoplasmic antibody MPO ANCA related with disease activity of vasculitis myeloperoxidase ( MPO ) specific anti neutrophil cytoplasmic antibodies ( MPO ANCA ) are involved in the development of vasculitis microscopic polyangiitis , a systemic vasculitis etc . We have showed a correlation of MPO ANCA epitopes in vasculitis concerning contribution of N and C terminus of MPO to severity of the diseases . On the other hand , a role of activated neutrophils in inflammatory nephritis renal lesions using SCG / Kj mice . In the phase of nephritis with a low grade of proteinuria , the spontaneous release of MPO from peripheral neutrophils increased , indicating that neutrophils are activated and contribute to the development of active crescentic lesions in SCG / Kj mice . In addition , we have investigated that mice having CADS / CAWS induced vasculitis also are good model animals for the analysis of the production of MPO ANCA . furthermore , we have clarified that MPO is a major antigen for MPO ANCA production using MPO knock mice .

Neutrophil fcgammariiib allelic polymorphism in anti neutrophil cytoplasmic antibody ( ANCA ) positive systemic vasculitis. (2000 Apr)
neutrophil fcgammariiib allelic polymorphism in anti neutrophil cytoplasmic antibody ( ANCA ) positive systemic vasculitis . neutrophils constitutively express fcgammariia and fcgammariiib receptors . Both receptors exhibit allelic variants which have different quantitative functional capacities : the biallelic fcgammariia R131 and H131 alleles , and the neutrophil antigen ( NA ) NA1 / NA2 alleles . ANCA activation of neutrophils requires ligation of fcgammariia receptor , but recent data have shown that ANCA can also bind fcgammariiib receptor . The aim of this study was to determine whether the fcgammariiib polymorphism was a risk factor for the development of ANCA associated systemic vasculitis , or the associated nephritis . fcgammariiib receptor genotyping was determined by allele specific polymerase chain reaction . genomic DNA was extracted from 101 caucasian patients with ANCA vasculitis ( of whom 84 had renal disease ) and 100 ethnically matched controls . Of the patients with ANCA systemic vasculitis , 71 had ANCA with specificity for proteinase 3 and 30 with specificity for myeloperoxidase ( MPO ) . overall no significant difference in genotype distribution or allele frequencies was found between patients and controls , or between patients with renal disease and controls . however , there was a trend for an increase in homozygosity for the NA1 allele in patients with a vasculitis and this was significant in patients who had anti MPO antibodies . The fcgammariiib receptor polymorphism is not a major factor predisposing to the development of ANCA systemic vasculitis or the associated …

Poor renal outcome of antineutrophil cytoplasmic antibody negative pauci immune glomerulonephritis in taiwanese. (2006 Sep)
Poor renal outcome of antineutrophil cytoplasmic antibody negative pauci immune glomerulonephritis in taiwanese . background / purpose : pauci immune glomerulonephritis ( GN ) is an important cause of crescentic GN , acute renal failure and mortality . however , data are limited on the clinical presentation and outcome in antineutrophil cytoplasmic antibody ( ANCA ) negative patients , especially in asians . methods : This retrospective study analyzed medical records and pathology slides of patients who received renal biopsy between february 1998 and october 2004 . enzyme linked immunosorbent assay was used routinely for ANCA testing in all patients . results : among 637 patients with biopsy proven GN included in this study , 88 ( 13 . 8 ) had glomerular crescent formation . among them , pauci immune crescentic glomerulonephritis ( picgn ) ( 42 patients , 47 . 2 ) and lupus nephritis ( 25 patients , 28 . 4 ) were the most common pathologic diagnoses . lupus patients were younger ( p 0 . 028 ) , while picgn patients had more chronic lesions ( p 0 . 001 ) , extensive glomerular crescents ( p 0 . 001 ) , less severe proteinuria ( p 0 . 001 ) and poorer renal survival ( p 0 . 0017 ) . among the picgn patients , 62 . 5 had a positive ANCA test , 80 had myeloperoxidase ANCA and 20 had proteinase 3 ANCA . subgroup analysis showed that ANCA negativity was associated …

Heat treatment of normal human sera reveals antibodies to bactericidal permeability inducing protein ( BPI ). (1999 Jul)
Heat treatment of normal human sera reveals antibodies to bactericidal permeability inducing protein ( BPI ) . Heat treatment of normal sera to 56 degrees C for 30 min , a common procedure for the inactivation of viruses , e . g . HIV , reveals the presence of antibodies to neutrophil cytoplasm antigens ( ANCA ) , as detected by indirect immunofluorescence on ethanol fixed human neutrophils and by antigen specific elisa for BPI . reactivity was not seen to the other common vasculitis associated antigens proteinase 3 ( PR3 ) or myeloperoxidase ( MPO ) . The effect of temperature was maximal at 56 degrees C , with substantial antibody demonstrable after only 5 min at this temperature . In experiments using polyethylene glycol ( PEG ) 6000 to remove immune complexes , the effect of heating could be abrogated by preincubation with 8 PEG , which suggested that these anti BPI antibodies might be complexed in sera . after passage of normal plasma over a protein G column , the acid eluted fraction contained elevated levels of antibodies to BPI but not to other vasculitis associated antigens such as PR3 or MPO , nor to glomerular basement membrane ( GBM ) , the goodpasture antigen which is recognized by the pathogenically important human antibodies shown to mediate nephritis in transfer experiments . moreover the levels of anti BPI in the IgG fraction could be augmented by preincubation with glycine pH 2 . 5 for 30 min . …

No association between neutrophil fcgammariia allelic polymorphism and anti neutrophil cytoplasmic antibody ( ANCA ) positive systemic vasculitis. (1999 Jul)
No association between neutrophil fcgammariia allelic polymorphism and anti neutrophil cytoplasmic antibody ( ANCA ) positive systemic vasculitis . ANCA , implicated as having a pathogenic role in systemic vasculitis , can activate tumour necrosis factor alpha ( TNF alpha ) primed neutrophils by cross linking surface expressed ANCA antigens with neutrophil fcgammariia receptors to release reactive oxygen species . The fcgammariia receptor exists as polymorphic variants , R131 and H131 , which differ in their ability to ligate human IgG2 and IgG3 . neutrophils homozygous for the fcgammariia H131 allotype bind more efficiently to IgG3 than the fcgammariia R131 allotype and are the only human fcgammar which bind IgG2 . Our aim was to determine whether the homozygous fcgammariia H131 individuals are more susceptible to developing ANCA associated systemic vasculitis and nephritis due to differential IgG binding and activation . fcgammariia allotype was determined by both allele specific polymerase chain reaction ( PCR ) and southern blotting with allele specific oligonucleotide probes end labelled with 32P gammaatp , after PCR amplification of genomic fcgammariia DNA in 107 caucasian patients with ANCA vasculitis ( of whom 89 had renal disease ) and 100 ethnically matched controls . phenotyping of neutrophil fcgammariia alleles was confirmed in some patients by quantitative flow cytometry using murine moabs 41h16 and IV . 3 . Of the patients with ANCA systemic vasculitis , 75 had ANCA with specificity for proteinase 3 and 32 with specificity for myeloperoxidase . overall , no skewing in fcgammariia allotypes …

Cavitating lung lesions in the course of ANCA associated vasculitis : differential diagnostic aspects antineutrophil cytoplasmatic antibodies ( ANCA ) … (1998 Aug)
cavitating lung lesions in the course of ANCA associated vasculitis : differential diagnostic aspects antineutrophil cytoplasmatic antibodies ( ANCA ) associated vasculitides ( wegener s granulomatosis , microscopic polyangiitis , churg strauss syndrome ) show quite variable courses . clinical features of the full blown generalized systemic vasculitis are usually found in the respiratory tract and the kidney . pulmonary involvement of wegener s granulomatosis shows commonly nodules and cavitations but also diffuse alveolar hemorrhage . We report the case of a 57 year old man suffering from dyspnea , thoracal pain , arthralgia , purpura , scleritis and tinitus . specimen of the kidney showed segmental glomerulosclerosis and tubulointerstitial nephritis . because of the presence of canca wegener s disease was assumed . pulmonary infiltrates developed under immunosuppressive treatment with cyclophosphamid . As differential diagnosis of the pulmonary infiltrates , we considered invasive pulmonary aspergillosis as well as infiltrates due to wegener s granulomatosis . In spite of maximal therapeutic management of patient died of respiratory and cardiovascular failure . The findings at autopsy showed distinct invasive pulmonary aspergillosis and perifocal hemorrhage .

ANCA GBM dot blot : evaluation of an assay in the differential diagnosis of patients presenting with rapidly progressive glomerulonephritis. (2004 May)
ANCA GBM dot blot : evaluation of an assay in the differential diagnosis of patients presenting with rapidly progressive glomerulonephritis . rapidly progressive glomerulonephritis ( RPGN ) is characterized by rapid and progressive loss of renal function and the presence of crescentic glomerulonephritis ( CGN ) . early diagnosis and appropriate treatment is mandatory to prevent death and / or renal failure . We have evaluated an ANCA GBM dot blot diagnostic test in terms of sensitivity , specificity , and inter observer effect in consecutive patients with RPGN ( n 82 ) . control sera ( n 34 ) included healthy and relevant disease controls . Dot blots were independently evaluated by nine observers . proteinase 3 ( PR3 ) ANCA , myeloperoxidase ( MPO ) ANCA , and both were detected by elisa in 36 , 32 , and 3 samples of 71 patients with pauci immune CGN , respectively . Two additional samples were ANCA negative . The dot blot revealed a sensitivity of 92 95 for PR3 ANCA and 80 86 for MPO ANCA . The specificity of the dot blot for PR3 and MPO ANCA was 100 . In the patients with anti GBM nephritis ( n 9 ) anti GBM was detected by both elisa and dot blot ( sensitivity : 100 ) . The specificity of the anti GBM dot blot was 91 94 . however , the inter observer effect was relatively high for detection of anti GBM antibodies ( 24 ) …

Contribution of myeloperoxidase in vasculitis development. (2004 Oct)
contribution of myeloperoxidase in vasculitis development . infiltrated neutrophils is believed to contribute to the progression of vasculitis . In particular , myeloperoxidase ( MPO ) specific antibodies against neutrophils , anti neutrophil cytoplasmic antibodies ( MPO ANCA ) is involved in the development of vasculitis microscopic polyangiitis etc . In japan a higher percentage of MPO ANCA than that in europe has been reported In addition , we showed a correlation of MPO ANCA epitopes of kawasaki disease patients by 47 with that of mothers . On the other hand , mice having CADS / CAWS induced vasculitis is a good model for the analysis of the production of MPO ANCA . We have clarified that MPO is a major antigen for MPO ANCA production using MPO KO mice . We also investigated the role of activated neutrophils in nephritis renal lesions using SCG / Kj mice . In the phase of nephritis with low grade of proteinuria , the spontaneous release of MPO from peripheral neutrophils increased , indicating that neutrophils are activated and contribute to the development of active crescentic lesion in SCG / Kj mice .

Anti neutrophil cytoplasmic antibodies ( ANCA ) in systemic lupus erythematosus : prevalence , clinical associations and correlation with other … (2005 Jan)
Anti neutrophil cytoplasmic antibodies ( ANCA ) in systemic lupus erythematosus : prevalence , clinical associations and correlation with other autoantibodies . AIM : This study was undertaken to clarify the nature of anti neutrophil cytoplasmic antibodies ( ANCA ) along with other autoantibodies in lupus nephritis ( LN ) patients and in systemic lupus erythematosus ( SLE ) patients without nephritis and to know their correlation with clinical manifestations and presence of other autoantibodies . material AND methods : fourty one LN patients and 18 SLE patients without nephritis were studied . LN patients were subdivided into diffuse proliferative glomerulonephritis ( DPGN ) , focal proliferative glomerulonephritis ( FPGN ) , rapidly progressive glomerulonephritis ( RPGN ) and membranoproliferative glomerulonephritis ( MPGN ) . Anti neutrophil cytoplasmic antibodies ( ANCA ) were detected by indirect immunofluorescence and confocal laser scanning microscope using PMN and HL60 cells . ANCA specificities like anti myeloperoxidase ( anti MPO ) , anti proteinase 3 ( anti PR3 ) , anti lactoferrin ( anti LF ) and anti cathepsin G ( anti CG ) were detected by elisa . other autoantibodies like anti nuclear antibodies ( ANA ) , anti double stranded DNA ( anti dsdna ) , anti single stranded DNA ( anti ssdna ) , anti ribonucleoproteins ( anti nRNP ) , anti smith antibodies ( anti Sm ) and rheumatoid factor ( RF ) were also tested . results : ANCA was detected in 37 . 3 patients . The predominant …