Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0042384 (vasculitis)
 20,525 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

In this study 23 cases of immune vasculitis with predominance of neurological symptoms were analysed. Besides patient history and neurological examination, myelotomy is the most relevant means of diagnosis in immune vasculitis. For this reason myelotomy should be integrated in routine diagnostic methods if the diagnosis immune vasculitis is discussed. Other laboratory parameters have no great relevance in the diagnosis of immune vasculitis. A higher specificity of antibody tests, especially in the case of ANA, might be able to replace myelotomy in the diagnosis of immune vasculitis. The relevance of laboratory parameters in the diagnosis of immune vasculitis in neurological patients was examined.
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PMID:Importance of laboratory clinical investigation in the diagnosis of immune vasculitis with neurological manifestation. 649 83

In a serological laboratory with a routine service for determining autoantibodies to human neutrophils, antibodies giving a selective or preferential reaction with the nucleus or perinuclear area of neutrophils are not uncommon. The aim of this study was to look for clinical correlates with the presence of such neutrophil-reactive autoantibodies. The specificity of such antibodies for nuclear or cytoplasmic antigens was studied in 65 consecutive sera displaying nuclear/perinuclear reactivity at a titre of at least 80 using the indirect immunofluorescence technique (IIF) on ethanol-fixed leucocytes. The sera were also investigated by IIF on formalin-acetone fixed leucocytes and on HEp-2 cells. ELISA techniques were used to measure antibodies to azurophil granule constituents (ANCA), purified myeloperoxidase (MPO-ANCA), and lactoferrin (LF-ANCA). Furthermore a qualitative spot immunoassay was used for the detection of antibodies to alpha, beta, and gamma fractions, and the nuclear fraction of neutrophils, purified proteinase 3 (PR3), MPO, enolase, lysozyme, elastase, lactoferrin, and cathepsin G. The diagnoses linked to such GS-ANA/pANCA positivity were arthritides, vasculitides, inflammatory bowel disease and chronic hepatic conditions. MPO was the main antigen recognized in the vasculitis group, but apart from that, rather limited antigen reactivity was demonstrable by these techniques, lysozyme being the most frequently recognized autoantigen in patients with arthritides. Human lymphocytes served as a suitable control substrate when distinguishing between GS-ANA/pANCA and ANA, whereas HEp-2 cells usually could not be used if both classes of antibodies were present in a sample. Furthermore, formalin-acetone fixation is not recommended for routine use.
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PMID:Clinical correlates and substrate specificities of antibodies exhibiting neutrophil nuclear reactivity--a methodological study. 749 88

In a 53-year-old female patient with recurrent, sometimes bloody diarrhea, the long standing diagnosis of an ANA-negative lupus erythematosus with membranoproliferative glomerulonephritis, leucocytoclastic vasculitis and chronic hepatitis was ruled out and the diagnosis of a hepatitis C associated cryoglobulinaemia was established. The origin of the diarrhea was due to intestinal vasculitis as a result of cold food or beverages.
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PMID:[Intestinal vasculitis and glomerulonephritis in hepatitis C- associated cryoglobulinemia]. 769 12

Pneumatosis cystoides intestinalis (PCI) is an uncommon disorder usually associated with intestinal and pulmonary obstructive diseases, recent abdominal procedures and systemic illnesses. PCI has been reported in patients with systemic lupus erythematosus associated with intestinal vasculitis. We describe herein a patient with a month history of intermittent abdominal pain, diarrhoea, hyporexia, and weight loss who underwent intestinal resection for acute abdomen. Post-operatively she gave a three-month history of arthritis of the right knee, ankles and feet, arthralgia of the wrists, MCPs and shoulders. She also described weakness, weight loss, Raynaud's phenomenon, and a skin rash. Laboratory examination revealed an increased ESR, low haemoglobin and haematocrit, positive rheumatoid factor, a positive ANA with a speckled pattern, as well antibodies to DNA, SS-A and cardiolipin. The abdominal symptomatology especially pain, cramps and bouts of diarrhoea persisted after the surgery and became worse two months later. Abdominal X-ray showed distention of bowel with cyst formation in the wall of the entire colon. A diagnosis of PCI was made radiologically. The intestinal pathology was reviewed and vasculitis was identified. The patient received treatment with high dose prednisone with an excellent response; prednisone was progressively tapered and she has been asymptomatic without abdominal complaints or other symptoms for over a year.
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PMID:Pneumatosis cystoides intestinalis in systemic lupus erythematosus with intestinal vasculitis: treatment with high dose prednisone. 808 81

Circulating antibodies to myeloperoxidase (MPO) have been described in a variety of vasculitic syndromes, drug-induced SLE and drug-induced nephritis. We have examined the autoantibody profile in acute sera from patients with antineutrophil cytoplasmic antibody-positive vasculitis (n = 8), drug-induced nephritis (n = 4), drug-induced lupus (n = 7), SLE (n = 27) and nephritis-associated with SLE (n = 17). Significant binding to purified MPO in ELISA was given by all sera from patients with vasculitis and drug-induced nephritis but ANA sought by indirect immunofluorescence on HEp-2 cells were not detected. Both anti-MPO and ANA were found in sera from patients with drug-induced lupus. Sera from patients with SLE or SLE nephritis did not contain high titres of anti-MPO antibodies but invariably contained ANA. Anti-MPO antibodies of both IgG and IgM classes were present in all sera from patients with drug-induced disease. Although the number of samples tested was small, sera from patients with drug-induced nephritis showed significantly greater median % binding of IgM to MPO compared with drug-induced SLE. Binding to MPO by IgG in these sera was not significantly different. These findings suggest that the mechanism of interaction between hydralazine and the immune system in the two drug-induced autoimmune diseases studied may contribute to their distinct clinical features.
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PMID:Autoantibodies to myeloperoxidase in idiopathic and drug-induced systemic lupus erythematosus and vasculitis. 816 72

A cutaneous or systemic vasculitis occurs in myelodysplasia as well as in myeloproliferative and lymphoproliferative disorders. The most common lesion is a leucocytoclastic vasculitis, with neurological or joint involvement occurring less often. The vasculitis may appear contemporaneously with or precede the clinical onset of the blood dyscrasia. Occasionally the lesions respond dramatically to the use of steroids but in general, patients with vasculitis have a worse prognosis than those with uncomplicated myelodysplasia. Vasculitis and myelodysplasia appear together too often for the association to be coincidental and the vasculitis in most cases cannot be attributed to intercurrent infections, therapeutic agents or a pre-existing rheumatological disorder. While autoantibodies are frequently present in myelodysplasia, and ANA and anti-neutrophil cytoplasm antibodies (ANCA) are found in other vasculitides, neither of these antibodies is associated with the vasculitis of myelodysplasia. There has however been one report of ANCA in Sweet's syndrome a non-vasculitic skin condition that also occurs in the myelodysplastic syndromes.
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PMID:Myelodysplasia, vasculitis and anti-neutrophil cytoplasm antibodies. 847 1

Antineutrophil cytoplasmic antibodies (ANCA) are important serological markers for the primary systemic vasculitides, including microscopic polyarteritis and necrotizing crescentic glomerulonephritis. Numerous reports have established the clinical utility of ANCA titer in monitoring disease activity, relapses, and response to treatment. ANCA, detected by indirect immunofluorescence (IIF) assays using patient's serum and ethanol-fixed human neutrophils, produce two common fluorescent staining patterns: cytoplasmic (C-ANCA), involving a 29-kD neutral serine protease termed proteinase 3 (PR3), and perinuclear (P-ANCA), the result mainly of myeloperoxidase (MPO), but occasionally by other components of the azurophilic granules including lysozyme, elastase, cathepsins, and lactoferrin. Some sera contain granulocyte-specific antinuclear antibodies (GS-ANA), which require formaldehyde fixation of neutrophils to cross link cytoplasmic antigens for distinguishing between ANCA and the GS-ANA by IIF. Positive IIF is confirmed by Western blot analysis or specific enzyme-linked immunosorbent assay for PR3, MPO, and other neutrophil granule antigens. The C-ANCA pattern is highly specific for Wegener's granulomatosis, a disease characterized by granulomatous inflammation, necrotizing and crescentic glomerulonephritis, and vasculitis; P-ANCA is found in sera of individuals with vasculitis, glomerulonephritis, and several other diseases. ANCA are predominantly immunoglobulin (Ig)G isotype, but may be IgM and IgA. Various pathophysiologic mechanisms have been proposed involving ANCA-mediated neutrophil activation in a hypothetical model of vasculitic diseases: positive signals via the FcgammaRII (CD32) receptor after IgG-ANCA binding to membrane-associated PR3, relevant cytokines, production of adhesion molecules on both activated neutrophils and endothelial cells, and the release of neutrophil reactive oxygen species and degranulation causing endothelial cell damage. Interference of C-ANCA with PR3 proteolysis and PR3 inhibition physiologically by the alpha1-proteinase inhibitor may have a pathogenic role. No convincing data have been reported for the existence of autoreactive T lymphocytes reactive to any degree with the neutrophil azurophilic enzymes. Studies of various drug- and infectious agent-related diseases and ANCA may contribute to understanding the mechanism(s) involved in some vasculitides.
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PMID:Antineutrophil cytoplasmic antibodies: major autoantigens, pathophysiology, and disease associations. 865 May 85

In this study of antineutrophil cytoplasmic antibody (ANCA)-associated diseases, we determined the prevalence of other autoantibodies and the antigen specificities of ANCA. ANA were common, occurring in 7 of 36 (19%) patients with Wegener's granulomatosis, in 16 of 34 (47%) patients with microscopic polyarteritis, in 6 of 11 (55%) patients with segmental necrotising glomerulonephritis and in 8 of 18 (44%) of those with ANCA-associated systemic vasculitis without renal involvement. ANA were associated more often with pANCA and microscopic polyarteritis than with cANCA (P < 0.05). Patterns were speckled (n = 23), homogeneous (n = 10) or nucleolar (n = 4). Anticardiolipin antibodies were also common, occurring in 10 of 25 (40%) patients with Wegener's granulomatosis, in 8 of 14 (57%) patients with microscopic polyarteritis and in 6 of 18 (33%) of those with a systemic vasculitis. However, anticardiolipin antibodies did not correlate with the presence of ANCA in any of the disease groups. Anti-GBM antibodies were demonstrated in only 2 of 25 (8%) patients with Wegener's granulomatosis, in 1 patient with microscopic polyarteritis (1/14, 7%) and in 1 with segmental necrotising glomerulonephritis (1/11, 9%). All four patients with anti-GBM antibodies had either cANCA or pANCA. In the second part of the study, the target antigens of ANCA were determined in Wegener's granulomatosis, microscopic polyarteritis, systemic vasculitis, inflammatory bowel disease, rheumatoid arthritis and systemic lupus erythematosus (SLE). Of the 19 sera with cANCA, 13 (68%) were directed against proteinase 3; other antigens were myeloperoxidase (1/19, 5%), elastase and lactoferrin together (1/19, 5%), lysozyme (1/19, 5%) or unknown (3/19, 16%). Of the 12 (58%) sera from patients with Wegener's granulomatosis who had cANCA, 7 bound to proteinase 3. Antimyeloperoxidase antibodies were present in 14 of 45 (31%) sera with pANCA; other antigens were proteinase 3 (5/45, 11%), elastase (3/45, 78%), lactoferrin (1/45, 2%), cathepsin G (5/45, 11%) or unknown (17/45, 38%). Antimyeloperoxidase antibodies were common in microscopic polyarteritis (6/14, 43%) and systemic vasculitis (5/16, 31%). However, the majority of target antigens in systemic vasculitis and rheumatoid arthritis with pANCA were not determined. "Atypical" ANCA were present in four patients, one with inflammatory bowel disease (1/8, 13%) and three with SLE (3/15, 20%). The specificities were cathepsin G, cathepsin G plus lactoferrin, or unknown in two sera. A recent report has suggested that bactericidal/permeability-increasing protein may be the target in patients with inflammatory bowel disease.
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PMID:Autoantibodies and target antigens in antineutrophil cytoplasmic antibody (ANCA)-associated vasculitides. 889 75

A 20-year old female patient with systemic lupus erythematosus with butterfly-erythema, arthritis, high positive ANA and anti-ds-DNA titres has to have laparatomy because of an acute abdomen. Histological diagnosis is a necrotizing vasculitis of the appendix. In systemic lupus erythematosus an intestinal vasculitis can be found which might be as severe as in polyarteritis nodosa.
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PMID:[Necrotizing vasculitis of the appendix and cecum in systemic lupus erythematosus]. 896 88

The aim of this work was the study of 88 patients with SLE for clinical, laboratorial and demographical parameters. The patients sera were assayed for the presence of anti-beta-2-microglobulin antibodies by ELISA. These spectrofotometric results were converted in a standard-deviation unity in relation to the average of 29 control individuals values. It was established a cut-off of 2.5 standard-deviation in order to determine the limit of normality as well as the cut-off of 10 standard deviation was chosen in order to defined the higher values. Several statistical significant associations were observed between the anti-beta-2-microgobulin and the clinical and laboratorial parameters. The clinical applications of those associations were determined by the predictive value. It was only considered the values greater then 75%. The negative predictive values found in the lower cut-off were 92% to ANA (homogeneous-peripheral pattern), 89% to high levels CIC, 81% to mucocutaneous vasculitis and 80% to depressed CH50 levels. The positive predictive values found in 88% to high level of mucoprotein, 83% to mucocutaneous vasculitis, 83% to the onset-disease-time lower six years and 78% to limphopenia. Special attention may be paid to the high sensibility and specificity of the anti-beta-2-microglobulin test to some of the parameters in the evaluation of the disease activity, mainly in mucocutaneous vasculitis.
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PMID:[Anti-beta-2-microglobulin antibodies in systemic lupus erythematosus]. 943 98


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