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Results for “Systemic Vasculitis”

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PubMed1987

Polyneuropathy and systemic vasculitis. An electrophysiological study.

A review of cases of systemic vasculitis followed for one year in a rheumatology department resulted in the detection of 18 patients who on the clinical and electrophysiological evidence had symmetrical distal polyneuropathy. A moderate impairment of the motor conduction velocity was present in 7 patients, with electromyographic neurogenic changes in the distal lower limbs of all but one patient. The sensory action potential of the sural nerve was bilaterally absent in one case, and its amplitude was reduced in 14 out of 16 patients, with a decreased sensory conduction velocity in 9 cases. The sural nerve biopsy, performed in 6 patients, was prevalently suggestive of previous axonal degeneration. This investigation illustrates the spectrum of diffuse peripheral nerve involvement associated with systemic vasculitis. A variable impairment of the sensory action potential, ranging from slight decrease of amplitude to no response, is the most common finding. Conduction velocities appear to be relatively spared.

Adult↗
PubMed1987

Association of alkaline phosphatase with an autoantigen recognised by circulating anti-neutrophil antibodies in systemic vasculitis.

A solid phase radioimmunoassay has been developed to detect circulating autoantibodies to neutrophil cytoplasmic antigens in systemic vasculitis. After fractionation of these antigens by size, with gel filtration high performance liquid chromatography, sera from patients with clinically different forms of systemic vasculitis, Wegener's granulomatosis (WG) and microscopic polyarteritis (MP), showed contrasting specificities of binding. WG sera bound to 100, 6.2, and 1.8 kD components, whereas MP sera bound only to the 100 kD component, allowing immunological distinction between the syndromes. The 100 kD component recognised by both WG and MP sera also showed alkaline phosphatase activity. Further evidence for this association was obtained by direct binding experiments between systemic vasculitis sera and calf-intestinal or human neutrophil alkaline phosphatase and by the cross-reactivity of W8, a monoclonal antibody raised to a neutrophil cytoplasmic autoantigen, with various preparations of the enzyme.

Alkaline Phosphatase↗
PubMed1996

T cell responses to myeloperoxidase (MPO) and proteinase 3 (PR3) in patients with systemic vasculitis.

T cell-mediated immune responses are likely to be important in the pathogenesis of systemic vasculitis. However, identifying the T cells involved has proved difficult, and there are conflicting reports regarding T cell proliferation in response to different autoantigens. Perinuclear (P) and cytoplasmic (C) anti-neutrophil cytoplasmic antibodies (ANCA) are closely associated with systemic vasculitis, and are generally specific for MPO or PR3, respectively. We studied the proliferative responses to MPO and PR3 of peripheral blood mononuclear cells from patients with P-ANCA or C-ANCA specific for these antigens by ELISA. These responses were compared with those of normal controls, and of disease controls with P- or C-ANCA not specific for MPO or PR3. The patient group as a whole showed significant T cell proliferation in response to the autoantigens compared with controls (P = 0.005). Cells from nine of 13 P-ANCA-positive, anti-MPO-positive patients proliferated in response to MPO, compared with five of 16 controls (P = 0.04). Cells from five of eight C-ANCA-positive, anti-PR3-positive patients proliferated in response to PR3, compared with two of 11 controls (P = 0.05). These experiments demonstrate that patients with P-ANCA or C-ANCA possess T cells which respond to MPO or PR3, respectively. As in other autoimmune diseases, responses to both antigens were also seen in a proportion of healthy controls. Further analysis of these responses will be important in understanding the pathogenesis of systemic vasculitis and in designing specific immunotherapy.

Autoantibodies↗
PubMed1991

Scleroderma and central nervous system vasculitis.

We describe a patient with scleroderma (CREST syndrome) and central nervous system vasculitis. While angiography demonstrated segmental symmetrical arterial narrowing characteristic of vasculitis, results of leptomeningeal biopsy were normal. There was no evidence of systemic vasculitis, renal failure, or malignant hypertension previously thought to be required to explain central nervous system dysfunction in patients with scleroderma. Signs and symptoms attributable to vasculitis were reversible with aggressive immunosuppressive therapy.

Cerebrovascular Circulation↗
PubMed1990

New strategies for plasma exchange in systemic vasculitis.

The use of plasma exchange in the treatment of systemic vasculitis has been controversial and poorly defined. Since the discovery of anti-neutrophil cytoplasm antibodies (ANCA) and the demonstration of their specificity for systemic vasculitis, there has been a reassessment of the use of plasma exchange along the same lines as its use in anti-glomerular basement membrane disease. ANCA has also contributed to the diagnosis and classification of rapidly progressive glomerulonephritis, in which there is now firmer evidence to support the use of plasma exchange. Current work into the immunoregulation of ANCA by idiotype/anti-idiotype interactions is leading to new approaches to therapy and plasma exchange alone appears capable of inducing prolonged remission in some patients with vasculitis, possibly through an effect on these interactions.

Autoantibodies↗
PubMed1990

Autoantibodies to endothelial cells and neutrophil cytoplasmic antigens in systemic vasculitis.

The interaction of circulating autoantibodies with the endothelium may be an important mechanism in the pathogenesis of systemic vasculitis. In a prospective study, we looked for circulating antiendothelial cell autoantibodies (AECA) and anti-neutrophil cytoplasm autoantibodies (ANCA) in 80 patients with suspected systemic vasculitis. AECA were measured using an isotype-specific cellular ELISA incorporating human umbilical vein endothelial cells. ANCA activity was determined by indirect immunofluorescence and radioimmunoassay. Sequential studies were performed on sera from four cases with dual positivity, where autoantibody binding was compared with von Willebrand factor (vWF) concentration and disease activity. IgG AECA were significantly higher in the 27 ANCA-positive sera as compared with normal controls (P = 0.027) with IgG (P = 0.009) and IgA (P = 0.046) AECA isotypes correlating with ANCA positivity; in contrast, no differences were found between AECA levels in the ANCA-negative sera and the normal controls. Cross-inhibition studies pointed to the co-existence of two autoantibody populations. An association between autoantibody binding, disease activity and vWF concentration was found for both ANCA and AECA. Some patients with systemic vasculitis have detectable AECA that recognize different epitopes to ANCA and like ANCA, their titre correlates with disease activity and thus they may have a pathogenetic role in these conditions.

Antibody Specificity↗
PubMed1985

Neurological complications of systemic vasculitis. A report of 2 cases.

In up to 72% of patients with systemic vasculitis the neurological system may be involved. This may be peripheral or central (brain or spinal cord), and is usually due to infarction or a ruptured aneurysm. Diagnosis and evaluation are based peripherally on examination of a biopsy specimen or centrally on angiography. Therapy is usually with steroids and/or immunosuppressive agents. Two patients with systemic necrotizing vasculitis with unusual neurological complications are presented.

Adult↗
PubMed1993

Pooled intravenous immunoglobulin in the management of systemic vasculitis.

Investigation into the therapeutic use of intravenous immunoglobulin (IVIg) in systemic vasculitis was prompted by the detection of anti-idiotype antibodies reactive with ANCA in IVIg and the proven ability of IVIg to reduce the incidence of coronary artery aneurysms in Kawasaki disease. The efficacy and safety of IVIg (Sandoglobulin) was assessed in an open study of 26 patients with active systemic vasculitis. Eight weeks after IVIg 13 patients were in full and 13 in partial remission, clinical benefit was maintained in 18 twelve months later and was reflected by changes in C-reactive protein and ANCA.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed2004

Endothelial cells, antineutrophil cytoplasmic antibodies, and cytokines in the pathogenesis of systemic vasculitis.

Endothelial cells play a pivotal role in the pathogenesis of systemic vasculitis. Endothelial cells have significant proinflammatory activities, amplifying and perpetuating the inflammatory process and contributing to vessel regeneration and repair. Significant contributions have improved the understanding of additional ways through which antineutrophil cytoplasmic antibodies (ANCA) may potentiate neutrophil- and monocyte-mediated endothelial cell activation and damage. Signaling pathways mediating ANCA effects have been delineated, and new animal models have demonstrated the pathogenic role of ANCA in the development of systemic vasculitis. Significant efforts have identified anti-endothelial cell antibody specificities and elucidated mechanisms through which these antibodies may promote endothelial cell activation and injury. New ways to assess in vivo endothelial cell damage and dysfunction also have been developed. In addition, besides being a relevant compensatory mechanism for ischemia, angiogenesis may have important proinflammatory functions in vasculitis. The potential relevance of bone-marrow-derived endothelial cell precursors in neovascularization has begun to be appreciated.

Animals↗
PubMed2004

[Pseudovasculitis--confusingly alike systemic vasculitis...but completely different treatment is required].

"Vasculitis mimicry" or "pseudovasculitis" are terms used for the description of a variety of disorders that simulate systemic vasculitis and often represent a challenge in daily rheumatological practice. These conditions may present with clinical, radiological, and laboratory features resembling those of systemic vasculitis. Nevertheless, the pathogenesis, therapeutic approach, and prognosis are different. The pseudovasculitides constitute an extremely heterogenous group of disorders with conditions caused by infections, malignancies, drugs and thromboembolism. A detailed history is mandatory and this should include dietary habits, concurrent medication, recent infections or invasive investigative procedures such as PTCA or conventional angiography. Incorrect diagnosis may lead to serious consequences due to delay of appropriate management and exposure of the patients to the risks of potentially toxic drugs. Thus, pseudovasculitis must be part of the differential diagnosis of patients presenting with manifestations of systemic vasculitis.

Adrenal Cortex Neoplasms↗
PubMed1989

Plasma concentrations of complement-activation complexes correlate with disease activity in patients diagnosed with isolated central nervous system vasculitis.

Isolated central nervous system (CNS) vasculitis is rare medium- sized vessel disease limited to intracerebral vessels. The two most common symptoms of this inflammatory disorder observed at entry to a hospital are headaches and mild memory deficits. Further progression of this disease may result in focal neurologic alterations and seizures. Currently, the most common laboratory abnormality noted is an elevated erythrocyte sedimentation rate. The complement (C) system is known to play a role in many inflammatory processes; it may also be involved in CNS vasculitis. In this longitudinal study of patients with CNS vasculitis, we detected C activation by highly sensitive and specific assays that are capable of identifying breakdown products formed after C activation: C3a des arg, C4a des arg, C5a des arg, C1rC1s-C1-inhibitor complex, and terminal C complex (C5b-9). We present two cases of documented CNS vasculitis in which serial measurements of these C-activation products correlate with disease activity. Our results indicate that a temporal association exists between C activation and the clinical presentation of CNS vasculitis. We conclude that physicians should monitor C-activation by-products in plasma when they attempt to follow the clinical course of CNS vasculitis.

Adult↗
PubMed1992

Treatment of antineutrophil cytoplasmic autoantibody-positive systemic vasculitis and glomerulonephritis with pooled intravenous gammaglobulin.

Antineutrophil cytoplasmic autoantibody (ANCA) is considered a serological marker for disease activity in patients with ANCA(+) systemic vasculitis. Recently, ANCA has been implicated as a pathogenic antibody that may be associated with neutrophil degranulation and release of lytic enzymes. Since intravenous gammaglobulin (IVIG) is known to contain antiidiotypic antibodies to ANCA, which could decrease the activity of the later, we chose to treat two patients with symptomatic ANCA(+) systemic vasculitis and glomerulonephritis with high-dose IVIG. The first patient, a 66-year-old man, developed rapidly progressive renal failure despite treatment with intravenous (IV) cyclophosphamide. The second patient, a 14-year-old boy, had relapsed 3 months after cessation of treatment with prednisone and cyclophosphamide. Both patients improved dramatically after treatment with IVIG, with the former recovering renal function within 11 days of therapy. In both patients, a concomitant reduction in serum ANCA titers was also observed. The second patient is currently in a sustained remission 14 months after his last IVIG dose on no other medication. These cases provide clinical evidence that IVIG has therapeutic benefit in modifying the immune-mediated injury associated with ANCA(+) systemic vasculitis and glomerulonephritis. In addition, IVIG may provide an additional safe therapeutic option to clinicians treating patient's with ANCA(+) vasculitis and glomerulonephritis who are not responsive to or are experiencing toxicity from conventional therapy.

Adolescent↗
PubMed1994

Autoantibodies to lactoferrin and histone in systemic vasculitis identified by anti-myeloperoxidase solid phase assays.

We aimed at confirming the antigen specificity recognized by anti-neutrophil cytoplasm antibodies (ANCA) in patients presenting systemic vasculitis with anti-myeloperoxidase (MPO) activity on ELISA. Thirty-five consecutive patients with reactivity in anti-MPO ELISA and systemic microscopic vasculitides were tested in slot and Western blot analyses. Eleven of 35 sera exhibited binding in Western blot studies with the MPO preparation used in the ELISA: five sera bound at the size of MPO, but five sera reacted with a 78 kD species (p78) co-purifying with MPO, and one serum blotted both MPO and p78. Sequence analysis and antigen-specific assays including Western blot studies showed that p78 is lactoferrin. All anti-lactoferrin positive sera, but no anti-MPO positive sera, also exhibited anti-nuclear binding on HEp2 cells with specificity for histone. We concluded that: (a) a subgroup of patients presenting systemic vasculitis with false anti-MPO reactivity on ELISA had anti-lactoferrin antibodies; (b) anti-lactoferrin was associated with anti-nuclear activity with specificity for histone; (c) these patients had systemic vasculitis without histological evidence of immune complex deposition.

Amino Acid Sequence↗
PubMed2000

Distribution of renal lesions in idiopathic systemic vasculitis: A three-dimensional analysis of 87 glomeruli.

Extracapillary proliferation and fibrinoid necrosis are the main diagnostic glomerular lesions in renal biopsy specimens of patients with idiopathic systemic vasculitis. Neither the incidence nor the correlation between extracapillary proliferation and fibrinoid necrosis in renal biopsy specimens from patients with systemic vasculitis has been systematically evaluated. By means of a three-dimensional analysis, we made a topographic reconstruction of the distribution of extracapillary proliferation and fibrinoid necrosis in affected glomeruli and tested different biopsy-processing protocols to optimize histopathologic analysis in clinical practice. Paraffin blocks of renal biopsy specimens from six patients diagnosed with systemic vasculitis were completely and serially sectioned in 2-microm thick sections and stained with the Gomori trichrome method. Glomeruli were scored per section for the presence of fibrinoid necrosis and extracapillary proliferation. Subsequently, a three-dimensional reconstruction was obtained for 87 glomeruli. In only one glomerulus did fibrinoid necrosis occur without extracapillary proliferation; in 51%, a combination of the two lesions was found; in 22%, extracapillary proliferation occurred in the absence of fibrinoid necrosis; and 26% did not show either lesion. Using the standard protocol from our department (ie, evaluation of 20 consecutive sections in various stainings), the chance of finding extracapillary proliferation was 100% and that of finding fibrinoid necrosis was 73%. If 5 sections stained with the Gomori trichrome were added, the latter percentage increased to 86%. Using skip-serial sections, even better results (87% to 92%) were obtained, with four skips as the best option (92%). In conclusion, our finding that fibrinoid necrosis rarely occurs in the absence of extracapillary proliferation may imply that both lesions are etiologically related. In addition, our observations indicate that the incidence of fibrinoid necrosis may be underestimated in clinical practice, depending on the number of sections evaluated.

Biopsy↗
PubMed1998

Outcome of renal replacement therapy in antineutrophil cytoplasmic antibody-associated systemic vasculitis.

Antineutrophil cytoplasmic antibody-associated systemic vasculitis (AASV) frequently leads to end-stage renal disease (ESRD). Potentially fatal disease activity can continue after the onset of ESRD in both dialysis and transplant patients, despite the immunosuppressive effects of uremia and rejection prophylaxis, leading to concerns that such patients have greater morbidity and mortality. To assess the outcome of AASV patients receiving renal replacement therapy, a retrospective analysis of 59 patients from our unit who received chronic dialysis, renal transplantation, or both, was performed. The survival of AASV patients with ESRD was comparable to national registry controls, as were both graft and patient survival after renal transplantation. Ther is no evidence that standard immunosuppressive protocols should be altered for AASV patients receiving renal transplants. The rate of relapse of vasculitis for patients on chronic dialysis and after transplantation was 0.09 and 0.02 per patient per year, respectively. These rates are lower than those of other series and support the contention that continued immunosuppression after ESRD, as practiced in our unit, is warranted. Relapses usually responded to cyclophosphamide and high-dose prednisolone treatment. Significantly, vasculitic flare-ups in dialysis patients were sometimes initially misdiagnosed as dialysis complications, leading to fatal delays in effective treatment. Follow-up by physicians experienced in the diagnosis and treatment of vasculitis activity should continue in these patients.

Adolescent↗
PubMed1999

Mechanisms of endothelial injury in systemic vasculitis.

The precise etiology of primary systemic vasculitis remains unknown. However, recent advances in the understanding of the role of and interaction between the vascular endothelium, mediators, and immune effector cells have helped to further elucidate those specific processes that result in endothelial cell damage. In WG and MPA, the evidence favors an autoimmune inflammatory response characterized by specific mediators in which the endothelium is both target and active participant.

Animals↗
PubMed1995

[Pneumocystis carinii pneumonia. Complications of cytotoxic treatment in a systemic vasculitis].

We report a patient with systemic vasculitis who developed a Pneumocystis carinii pneumonia whilst he was on steroid n cyclophosphamide treatment. Severe lymphopenia was noted with a CD4 count of 0.023 x10(9)/1 with no evidence of Human Immunodeficiency virus infection. A literature review of recent reports about this particular topic reveals that the development of lymphopenia in patients under immunosuppressor treatment is a fact of a paramount importance. In that sense, a periodic monitorization of both the granulocyte and the lymphocyte count is widely recommended in patients under cytotoxic treatment. A dose adjustment or even suppression of drug administration would be necessary if severe lymphopenia was observed.

Aged↗