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Results for “Systemic Vasculitis”

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PubMed1998

Systemic vasculitis. Diagnostic clues to this confusing array of diseases.

Systemic vasculitis can be difficult to recognize because of the many types of the disease and the conditions that can mimic it. However, early identification and initiation of treatment (often empirical) are important to avoid severe morbidity. In this symposium article, Dr Bush clearly describes the most common types of vasculitis according to current nomenclature based on the size of affected vessels. Illustrations of common signs of the various types of vasculitis are also included.

Humans↗

PubMed1995

ANCA and predicting relapse in systemic vasculitis.

We studied 60 patients with ANCA-positive systemic vasculitis (SV) to assess the prognostic significance of clinical and serological features at presentation, and the value of sequential monitoring of ANCA, C-reactive protein (CRP) and ESR levels as predictors of disease relapse. Patients were recruited at the time of diagnosis, treated with a standard protocol, and assessed monthly for one year. Clinical remission was achieved in 56/60 (93%), and ANCA became undetectable in 50/60 (83%) after treatment. During the one year follow-up period, disease relapses were seen in 23 (38%) patients. No specific associations were observed between initial disease presentation, initial ANCA level or ANCA antigenic specificity and relapse. However, 13/23 (57%) of relapses were preceded by a rise in ANCA a mean of 7.8 weeks earlier, while at the time of relapse 19/23 (83%) were ANCA-positive. Rises in CRP and ESR occurred in 23/60 (38%) and 14/43 (33%), respectively, but were less closely associated with relapse than ANCA. A sustained rise in ANCA was seen in six patients without relapse while clinical relapse occurred with a negative ANCA in four. Sequential ANCA monitoring at monthly intervals during disease remission is of value, at least during the first year, in the prediction and diagnosis of relapse in SV, and is superior to measurement of CRP or ESR.

Antibodies, Antineutrophil Cytoplasmic↗

PubMed1996

Etoposide: more effective and less bone-marrow toxic than standard immunosuppressive therapy in systemic vasculitis?

In two patients suffering from ANCA-positive systemic vasculitis (one from Wegener's granulomatosis, the other from microscopic polyangiitis), who were both resistant to or could not tolerate standard immunosuppressive therapy, complete clinical and biochemical remission was obtained within few months, treating with cyclic etoposide. Etoposide therapy was found to be significantly less bone marrow toxic than standard immunosuppressive therapy.

Adolescent↗

PubMed2001

New approaches to treatment in systemic vasculitis: biological therapies.

Although the effectiveness of biological agents in systemic vasculitis is unproven, their introduction heralds a new era of vasculitis treatment. These agents offer the promise of targeted immunotherapies; the possibility of greater efficacy (and fewer side-effects) than conventional vasculitis treatments; and the potential to provide novel insights into the pathophysiology of these diseases-insights that may be gained only by using these agents in humans. Challenges to the investigation of these therapies in the systemic vasculitides exist, but important basic and clinical investigations are already in progress. We review the major issues facing the investigation of biological agents in vasculitis; examine the rationale for believing that biological strategies in vasculitis will be efficacious; identify several candidate targets for biological approaches; and discuss the results to date of early studies. The potential biological targets discussed include cytokines such as tumour necrosis factor; interleukins-1, -6, and -12; interferon-gamma; the co-stimulatory molecules B7-1 and B7-2; and others.

Abatacept↗

PubMed2004

Infliximab improves endothelial dysfunction in systemic vasculitis: a model of vascular inflammation.

BACKGROUND: Endothelial vasomotor dysfunction and markers of systemic inflammation are independent determinants of cardiovascular risk. However, the link between clinical inflammation and endothelial dysfunction is unclear. The aim of this study was to use anti-neutrophil cytoplasmic antibody-associated systemic vasculitis (AASV) as a model of systemic inflammation in which to test the hypothesis that inflammation is associated with endothelial dysfunction and can be reversed with anti-tumor necrosis factor-alpha (TNF-alpha) therapy. METHODS AND RESULTS: Fourteen patients with active AASV and 21 age-matched control subjects were studied. Endothelial function was assessed through the use of forearm plethysmography and related to clinical disease activity: Birmingham Vasculitis Activity Score (BVAS) and serum levels of C-reactive protein (CRP), interleukin-6 (IL-6), and TNF-alpha. The effects of anti-TNF-alpha therapy (infliximab), either alone (n=6) or in combination with standard treatment (n=4), on endothelial function were subsequently determined. Patients had a mean BVAS of 11+/-1, and CRP and IL-6 were higher in the AASV group than in control subjects (34.8+/-10.5 versus 1.6+/-0.2 pg/mL, P<0.001; 9.0+/-0.7 versus 6.7+/-0.6 pg/mL, P=0.02). Forearm blood flow response to acetylcholine (ACh) was reduced in the patients compared with control subjects (P=0.002), but sodium nitroprusside (SNP) responses were not (P=0.3). The response to ACh improved with infliximab treatment (P=0.004) in particular, with infliximab alone (P=0.03). CONCLUSIONS: AASV is associated with endothelial dysfunction. Anti-TNF-alpha therapy, alone or in combination with standard treatment, results in clinical remission, reduced inflammation, and improved endothelium-dependent vasomotor responses.

Anti-Inflammatory Agents, Non-Steroidal↗

PubMed1996

Circulating intercellular adhesion molecule 1 in rheumatoid arthritis--relationship to systemic vasculitis and microvascular injury in nailfold capillary microscopy.

Intercellular adhesion molecule-1 (ICAM-1) is a membrane bound molecule that plays an important role in the pathogenic inflammatory responses observed in vasculitis. The aim of this study was to determine whether levels of soluble ICAM-1 sICAM-1) shedding into the circulation reflect the vascular injury found in nailfold capillaroscopy as well as systemic vasculitis in RA patients. We determined serum levels of sICAM-1 and soluble interleukin-2 receptor (sIL-2R) by an enzyme-linked immunosorbent assay in 79 RA patients. Serum levels of sICAM-1 were significantly increased in RA patients compared to 30 healthy controls. RA patients with clinical signs of systemic vasculitis showed significantly higher levels of sICAM-1 than those without vascular involvement. Although no significant correlation between sICAM-1 levels and the capillaroscopy findings were found, 75% of the patients with severe vascular changes in capillaroscopy exceeded normal sICAM-1 cut off value. Serum sICAM-1 concentrations correlated significantly with the erythrocyte sedimentation rate and serum sIL-2R, but not with the duration of RA, radiological stages, Ritchie index, age or type of treatment. These findings suggest that increased levels of sICAM-1 in serum of RA patients reflect systemic vascular involvement rather than a local vascular injury.

Arthritis, Rheumatoid↗

PubMed2001

Prolonged prodrome, systemic vasculitis, and deafness in Cogan's syndrome.

Cogan's syndrome is a rare, multisystem disease which occurs predominantly in children and young adults. It was originally described as the combination of interstitial keratitis and audiovestibular disturbance, but other forms of ocular disease, as well as systemic vasculitis, have since been recognised as part of the syndrome. Diagnosis can be difficult if the various manifestations occur separately, but early recognition is important because prompt treatment may prevent deafness. Two cases are presented here illustrating the features of this disease, and providing histological evidence of systemic vasculitis in both.

Adult↗

PubMed1999

[An autopsy case of Goodpasture's syndrome with P-ANCA and systemic vasculitis].

A 72-year-old woman was admitted because of anorexia and dyspnea. She was given a diagnosis of pulmonary hemorrhage and renal failure. Despite treatment with high-dose steroid and hemodialysis, the patient died of disseminated intravascular coagulation on the 9th hospital day. Autopsy revealed intra-alveolar hemorrhage, crescentic glomerulonephritis, and systemic vasculitis with fibrinoid necrosis. A direct immunofluorescence study demonstrated linear deposition of IgG along the glomerular basement membrane (GBM). Both anti-GBM antibody and anti-neutrophil cytoplasmic antibody with perinuclear pattern (P-ANCA) were detected in the patient's serum by enzyme immunoassay. Goodpasture's syndrome with P-ANCA was diagnosed. There has been some controversy as to whether vasculitis occurs in patients with Goodpasture's syndrome. This was a rare example of well-documented Goodpasture's syndrome with P-ANCA and systemic vasculitis, the exact etiologic relationships among which remain to be clarified.

Aged↗

PubMed1997

Antibody reactivity against thyroid peroxidase and myeloperoxidase in autoimmune thyroiditis and systemic vasculitis.

Potential cross-reactivity between thyroid peroxidase (TPO) and myeloperoxidase (MPO) molecules was evaluated by analysing the binding of 199 TPO antibody- and MPO antibody-positive sera to TPO and MPO molecules. Sera from six patients with autoimmune thyroiditis (AITD) and four patients with systemic vasculitis (SV) with different TPO-MPO antibody findings were then chosen for further analyses. All six patients with AITD had TPO antibodies in enzyme immunoassay (EIA) and four of them had simultaneously MPO antibodies in EIA. In AITD patients antibody binding to TPO could not be inhibited by adding native MPO to the serum diluent, suggesting that the possible cross-reactive epitopes were exposed in the denaturated MPO molecule. Similarly, the MPO ab reactivity of patients with systemic vasculitis could not be inhibited by native TPO. To study whether TPO and MPO antibodies recognize linear epitopes, the binding of antibodies to synthetic TPO and MPO peptides was analysed. Several TPO and MPO peptides were reactive, including peptides reacting with both TPO and MPO antibody-positive sera. One of the most cross-reactive peptides contained AA 586-601 in TPO, showing also particularly high AA homology (88%) with MPO (AA 594-609). The results suggest that TPO and MPO molecules contain cross-reactive epitopes that are exposed in denaturated molecules and may thus cause false positive antibody findings in solid phase EIA assays.

Amino Acid Sequence↗

PubMed1993

Pro- and anti-inflammatory cytokines in primary systemic vasculitis.

The expression of cytokines that are potentially involved in the pathogenesis of vasculitis was studied in patients with primary systemic vasculitis (PSV). In extension of earlier reports, we detected an overexpression of transforming growth factor beta (TGF beta), interleukin 6 (IL6), and interleukin 8 (IL8), indicating that the whole cytokine cascade is activated to a significant extent in PSV.

Churg-Strauss Syndrome↗

PubMed1975

The spectrum of systemic vasculitis: a classification to aid diagnosis.

Determining the cause of systemic vasculitis can be facilitated by a clinicopathologic classification of the syndrome. The considerations in differential diagnosis include periarteritis nodosa, leukocytoclastic angiitis (hypersensitivity angiitis, Schonlein-Henoch purpura, other disease-associated vasculitis), Wegener's granulomatosis, allergic granulomatosis (granulomatous angiitis), and giant cell arteritis.

Arteritis↗

PubMed1982

Circulating immune complexes in cutaneous and systemic vasculitis: clinicopathological correlations.

A high incidence of raised levels of circulating immune complexes has found in a group of patients with idiopathic vasculitis. Serial measurements in 8 patients with systemic vasculitis showed a good correlation between clinical status and circulating immune complex levels. Some correlation existed between the constituents of circulating and tissue-bound complexes in 6 patients so studied. However, there was no correlation between apparent organ involvement and hypocomplementaemia and the levels of size of circulating immune complexes. It is concluded that the measurement of circulating immune complexes may be clinically useful in vasculitis, but that no direct evidence is yet available that they are pathogenetically related to those which are tissue-bound.

Antigen-Antibody Complex↗

PubMed1985

Phenylbutazone-induced systemic vasculitis with crescentic glomerulonephritis.

Two patients had phenylbutazone-induced systemic vasculitis syndrome. Both presented with acute oliguric renal failure, and renal biopsies showed severe crescentic glomerulonephritis with marked interstitial inflammatory cell infiltration. Withdrawal of phenylbutazone and treatment with immunosuppressives and plasma exchange led to recovery of renal function in one case.

Acute Kidney Injury↗

PubMed1995

Management of systemic vasculitis: contribution of scintigraphic imaging to evaluation of disease activity and classification.

The use of radio-isotope-labelled leucocyte scans has become established as a non-invasive and accurate means of diagnosing a variety of inflammatory conditions. We report a retrospective study on leucocyte imaging in the management of 50 patients with systemic vasculitis. Leucocyte imaging was useful for detecting unsuspected sites of disease and monitoring disease activity. Scintigraphy was superior to conventional radiography or CT scanning for detecting and monitoring vasculitic involvement of the respiratory tract. The scans were useful for differentiating between Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA). There was a close and statistically significant relationship between the clinical diagnosis of WG and nasal uptake on leucocyte scans (p < 0.01), whereas in patients with MPA it was rare. Anti-proteinase 3 autoantibody specificity correlated significantly with nasal uptake of labelled leucocytes (p < 0.03). Leucocyte imaging is a useful non-invasive investigation in patients with systemic vasculitis.

Autoantibodies↗

PubMed1994

Autoantibodies against endothelial cells, extracellular matrix, and human collagen type IV in patients with systemic vasculitis.

Endothelial cells and subendothelial matrix (ECM) are involved in the pathogenesis of vasculitis. Exposure of the ECM following vascular damage may promote further immune and inflammatory response. To investigate this, we studied the prevalence of antibodies against endothelial cells (AECA), ECM, and its major component collagen type IV in systemic vasculitis patients. Seventy-one percent of patients had AECA (binding index, means +/- SD: 64.8 +/- 48.1%; normal controls: 8.9 +/- 6.9%, P < 0.001). Anti-ECM and anti-collagen type IV antibodies were also significantly higher in patients compared to normals (anti-ECM: 28.6 +/- 29.6% vs 9.0 +/- 11.3%, P < 0.002; anti-collagen type IV: 23.5 +/- 20.3% vs 8.1 +/- 9.1%, P < 0.002). AECA correlated with anti-ECM (r = 0.75, P < 0.0001) but not with anti-collagen type IV. Anti-ECM correlated with anti-collagen type IV (r = 0.45, P < 0.01). Positivity of cytoplasmic anti-neutrophil cytoplasmic antibodies (cANCA) was significantly lower in patients positive for anti-ECM and/or anti-collagen type IV antibodies (58% vs 11%, P = 0.048). AECA binding was partially reduced with ECM incubation by 25.1%. The addition of heparin caused a dose-dependent inhibition of binding activity (19.2-30.6%) in the AECA ELISA. These results support the hypothesis that there is a humoral response against ECM components in addition to endothelial cells in systemic vasculitis patients which might have pathological significance in vascular damage.

Adult↗

PubMed1993

Miscellaneous vasculitic syndromes including Behçet's disease and central nervous system vasculitis.

Behçet's disease is a chronic, relapsing, inflammatory process with clinical features of aphthous oral and genital ulcerations, uveitis, cutaneous vasculitis, arthritis, and central nervous system involvement. Large-vessel vasculitis, thrombophlebitis, and gastro-intestinal ulcerations may occur. As in other rheumatologic diseases, a genetic predisposition coupled with a triggering event leads to alterations in immune function and response, with variable clinical disease manifestations. Mononuclear cell activation and cytokine release, neutrophil chemotaxis, and humoral immune response with elevations of circulating immune complexes contribute to the pathogenesis of Behçet's disease. Vasculitis and vascular involvement are highlighted. There is no single diagnostic test for Behçet's disease. Treatment is comprised of anti-inflammatory and immunosuppressive agents and, in selected individuals, anticoagulation. Isolated central nervous system vasculitis or noninfectious primary (granulomatous) angiitis of the central nervous system is reviewed briefly. This condition is rare, predominantly affects young women, and includes symptoms of headache, weakness, and confusion. Characteristically, erythrocyte sedimentation rate is normal, serologic studies are negative, and cerebral angiography shows multifocal and segmental vascular narrowing and beading. Typical histopathology is granulomatous in less than 50% of patients, followed by necrotizing or lymphocytic vasculitis, or both, often with thrombosis. Treatment with immunosuppressive agents improves symptoms and outcome.

Behcet Syndrome↗

PubMed1995

Peripheral blood T lymphocytes in systemic vasculitis: increased T cell receptor V beta 2 gene usage in microscopic polyarteritis.

Antigen recognition by T lymphocytes is mediated by cell surface receptors T cell specificity depends on the variable, diversity and junctional (VDJ) regions of the alpha and beta polypeptide chains of the T cell receptor (TCR). The expression of the variable region genes of the beta chain (V beta) has been analysed to study the involvement of peripheral blood T cells in systemic vasculitis. RNA was extracted from peripheral blood lymphocytes of 12 patients with microscopic polyarteritis, 10 with Wegener's granulomatosis, six with unclassified vasculitis, and 28 healthy age- and sex-matched individuals. Complementary DNA was made from RNA and amplified by the anchored polymerase chain reaction (PCR) using redundant oligonucleotide primers for the TCR V beta genes. To determine if the dominant usage of a V beta gene family reflected the presence of particular T cell clones, cDNA was amplified with primers for the specific V beta gene family. The product was screened for sequence homogeneity by single-stranded conformational polymorphism (SSCP) and cloned to sequence the adjoining TCR (D beta) J beta region. A significant increase in the mean percentage expression of the V beta 2.1 gene was seen in vasculitis patients (11.4 + 1.0% (mean + s.e.m.)) compared with controls (6.6 + 0.6%; P < 0.003). The most marked increase was seen in microscopic polyarteritis (13.9 + 1.7%; P < 0.0001). There were also increases in the expression of V beta 3, 13 and 14 in peripheral blood of vasculitis patients compared with controls. SSCP analysis of V beta 2.1 amplified products indicated the presence of oligoclonal bands in a smaller proportion of patients (8/27) than controls (12/28). There was no strong evidence for the conservation of the TCR V beta 2.1 junctional region sequence data from a sample group of three patients with oligoclonal bands. Thus, a subset of patients with systemic vasculitis, particularly those with microscopic polyarteritis, have increased TCR V beta 2.1 gene expression in their peripheral blood T cell repertoire. As superantigens binding V beta 2.1 are postulated to activate T cells with diverse CDR3 sequences, it is proposed that a superantigen is involved in the immunopathogenesis of vasculitis.

Adult↗

PubMed1989

Sequential development of systemic vasculitis with anti-neutrophil cytoplasmic antibodies complicating anti-glomerular basement membrane disease.

Anti-neutrophil cytoplasmic antibodies (cANCA) were detected in 3 patients with anti-glomerular basement membrane (anti-GBM) disease. All 3 cases presented late in their clinical course, with severe renal involvement and alveolar hemorrhage. Anti-neutrophil cytoplasmic antibodies were associated with clinical features outwith the lungs and kidneys; in one case cANCA were initially absent but subsequently developed concurrently with the clinical appearance of systemic vasculitis as the anti-GBM antibody titer was falling. These findings confirm that cANCA can complicate anti-GBM disease and suggest that cANCA may identify a distinct subset of patients with anti-GBM disease, supporting a pathogenic role for cANCA in the development of systemic vasculitis.

Anti-Glomerular Basement Membrane Disease↗