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PubMed2003

[Therapy of primary systemic vasculitis].

The treatment of systemic vasculitides has changed during the past ten years from a uniform immunosuppressive regimen consisting of cyclophosphamide and prednisolone to a stage- and activity-adapted, more differentiated immunosuppressive and immunomodulating therapy. Due to the low incidence of these diseases, the assessment of these regimens by prospective, randomized, controlled trials has only been enabled through multicenter cooperation. In primary systemic vasculitides of small- or medium-sized vessels induction of remission therapy comprises methotrexate in early generalized disease without significant renal insufficiency, cyclophosphamide in full-blown generalized disease and additional plasma exchanges in cases of rapidly progressive glomerulonephritis if oliguric, each in conjunction with oral prednisolone. After achievement of remission patients are switched to a less potent maintenance of remission therapy with better long-term tolerability, such as azathioprine. Glucocorticoids are the mainstay for large vessel vasculitides, supplemented by azathioprine or methotrexate in cases of critical organ perfusion or demand for high steroid doses. Therapy for secondary vasculitides relies essentially on the treatment of the underlying disease, which means virus elimination in cases of hepatitis B or C associated vasculitides. Immunosuppression in these diseases is reserved for organ- or life-threatening manifestations only.

Adjuvants, Immunologic↗
PubMed1986

Peripheral neuropathy in systemic vasculitis: clinical and electrophysiologic study of 22 patients.

Twenty-two patients with evidence of systemic vasculitis and peripheral neuropathy were clinically and electrophysiologically investigated in a retrospective study. Ten had a polyarteritis nodosa, 6 a probable polyarteritis nodosa, and 6 a Churg-Strauss syndrome. Nine patients presented clinically with mononeuropathy or mononeuropathy multiplex considered typical of ischemic involvement of peripheral nerve; nine had more diffuse neuropathy, two of them a symmetric polyneuropathy. EMG examination revealed more diffuse neuropathy than clinically predicted. Findings were of acute or subacute axonal neuropathy.

Adult↗
PubMed2001

Antineutrophil cytoplasm autoantibodies from patients with systemic vasculitis activate neutrophils through distinct signaling cascades: comparison with conventional Fcgamma receptor ligation.

In systemic vasculitis, interactions between antineutrophil cytoplasm autoantibodies (ANCAs) and neutrophils initiate endothelial and vascular injury. ANCAs directed against either myeloperoxidase (MPO) or proteinase 3 (PR3) can activate cytokine-primed neutrophils by binding cell surface-expressed MPO or PR3, with the concurrent engagement of Fcgamma receptors (FcgammaR). Because roles for phospholipase D (PLD) and phosphatidylinositol 3 kinase (PI3K) have been demonstrated in FcgammaR activation of neutrophils, this study investigated the hypothesis that ANCA stimulation of neutrophils involved a similar engagement of FcgammaR and activation of PLD and PI3K. Pretreatment of tumor necrosis factor (TNF) alpha-primed neutrophils with antibodies against FcgammaRII and FcgammaRIII inhibited MPO-ANCA and PR3-ANCA induced superoxide generation, confirming that FcgammaR ligation is involved in ANCA-mediated neutrophil activation. However, although stimulation of TNF-alpha-primed neutrophils by conventional FcgammaR ligation, either using antibody-mediated cross-linking of FcgammaR or aggregated IgG, induced PLD activation, ANCA stimulation did not. Moreover, although ANCA-induced neutrophil activation results in significant PI3K activation-as assessed by phosphatidylinositol 3,4,5-triphosphate generation-conventional FcgammaR ligation, but not ANCA, activates the p85/p110 PI3K subtype. Inhibition of ANCA-induced superoxide generation with pertussis toxin suggests that ANCAs activate the p101/p110gamma PI3K isoform. In addition, the kinetics of activation of protein kinase B differs between conventional FcgammaR ligation and ANCA stimulation of neutrophils. These results demonstrate that though ligation of FcgammaRIIa and FcgammaRIIIb may be necessary, it is likely that ANCAs require other membrane cofactors for neutrophil activation.

Androstadienes↗
PubMedDate not supplied

[Isolated central nervous system vasculitis manifesting as a pseudotumor].

Vasculitis is characterized by inflammation of the vessel walls, with or without necrosis, that causes narrowing and occasionally obstruction of the vessels. Vasculitis can be a primary process or it can occur secondary to other disease. Isolated central nervous system vasculitis (ICNSV) is a rare type of primary vasculitis that predominately affects the supratentorial regions. The clinical presentation and imaging findings are unspecific and diagnosis is made by exclusion and biopsy. We present a case manifesting as a pseudotumor in the posterior fossa on computed tomography and magnetic resonance imaging. The diagnosis was after biopsy, and the patient evolved favorably after treatment.

Adult↗
PubMed1993

Relapses in patients with a systemic vasculitis.

Treatment with cyclophosphamide and steroids has greatly improved survival in patients with systemic necrotizing vasculitis but does not always provide a complete cure. There are as yet few data on the incidence, pattern and outcome of relapses in these diseases. We studied relapses in 150 consecutive patients with an idiopathic necrotizing vasculitis: 12 with classical polyarteritis (CPAN); 95 with microscopic polyarteritis (MPA); 28 with Wegener's granulomatosis (WG); and 15 with limited Wegener's granulomatosis (LWG). The relapse rates and median time to relapse in months were: CPAN, 41.7%/33; MPA, 25.4%/24; WG, 44%/42; LWG, 52%/18. The clinical features of relapse were similar to or more aggressive than those of the original presentation in CPAN and LWG and included renal disease for the first time, but in MPA and WG, relapse involved less renal involvement in the majority of cases. Laboratory tests, although often positive at relapse, were unhelpful in its prediction. The considerable accumulative non-fatal relapse rate contrasts with the very good long-term survival rates, and confirms the importance of long-term follow-up in systemic vasculitis.

Adult↗
PubMed1985

[Asthma, eosinophilia and systemic vasculitis: Churg-Strauss syndrome].

In two female patients asthma, marked eosinophilia and symptoms of systemic vasculitis could be classified clinically or histologically as allergic angiitis and granulomatosis (Churg-Strauss syndrome). The course in one patient went back over 20 years, characterized by an asymptomatic interval of 16 years. It is to be assumed that the disease is more frequent than it is diagnosed. It has to be thought of when, in addition to asthma and eosinophilia, there is evidence of involvement of other organs.

Asthma↗
PubMed1997

Chronic immunosuppressive therapy for systemic vasculitis.

Treatment with glucocorticoids and cytotoxic agents has markedly improved the outcome in patients with systemic vasculitis. As more patients survive with these disorders, the long-term risks of these therapies have become increasingly apparent. An understanding of therapeutic toxicities is important because in some instances they can be minimized by the use of treatment strategies and monitoring techniques. Pneumocystis carinii pneumonia is associated with a high mortality rate in immunosuppressed patients but can be prevented by prophylaxis. Glucocorticoid-related avascular necrosis can incur morbidity affecting employment and daily function. Although treatment options remain limited, investigation into joint salvage procedures has continued. The frequency and longevity of urologic toxicity associated with cyclophosphamide therapy have recently been addressed. Identification of this risk has led to the development of long-term monitoring strategies in cyclophosphamide-treated patients.

Cyclophosphamide↗
PubMed1989

[An unusual slow course of systemic vasculitis with multiorgan involvement].

This paper reports on an unusually slowly progressing course seen in systemic vasculitis of the median and smaller arteries and small veins associated with dermatomyositis, chronically active hepatitis, glomerulonephritis of the immune complex and restrictive cardiomyopathy in a 35-year-old patient. The authors discuss the difficulties in establishing the diagnosis and in finding the exact classification of this disease.

Adult↗
PubMed1986

[Various aspects of the pathogenesis of systemic vasculitis].

Based on the reported data and observation of 86 patients with different forms of systemic vasculitis (SV) the authors analyzed the leading pathogenetic mechanisms of generalized vascular injuries seen in these diseases. The data presented attest to the role of disorders of immunoregulation, immune complex reactions, an increase in the blood coagulation potential, a rise of platelet and red cell aggregation in the pathogenesis of the main clinical syndromes of SV. At the same time the authors describe in detail the microcirculatory disorders seen in the main disease entities and discuss the concrete reasons for the development of capillarotrophic insufficiency and marked tissue hypoxia. Close interaction of the main pathogenetic factors of SV and the character of the disorders determine the necessity of a differentiated approach to the problems of the treatment of patients depending on the disease entity and the leading pathogenetic components.

Autoimmune Diseases↗
PubMedDate not supplied

The eye in systemic vasculitis.

The term 'vasculitis' includes a wide range of disorders characterised by inflammation of the wall of blood vessels, sometimes with necrosis, leading to ischaemia of the affected organ. The exact pathogenesis of most of these vasculitides is not fully understood and although the presence of circulating auto-antibodies seems to be a common feature among them, each vasculitis has its unique pathogenesis and a predilection for vessels of a defined size. Systemic vasculitis may be associated with ocular complications which include scleritis, keratitis, uveitis and optic neuropathy. These can precede the symptoms/signs of the systemic disease and therefore their recognition may lead to detection of the underlying disorder. The eye may also be affected by the treatment required to control the systemic disease.

Autoimmune Diseases↗
PubMed1997

Biopsy diagnosis of systemic vasculitis.

A definitive diagnosis of virtually all vasculitides requires histological documentation. Although each major type of systemic vasculitis may have its own unique features, variability and overlaps still exist, and histopathological specificity is rarely an absolute discriminator. The interpretation of biopsies for the diagnosis of vasculitis remains more an art than a science, and it requires full and complete correlation with historical, clinical, laboratory, and angiographic findings.

Biopsy↗
PubMed1991

Anti-neutrophil cytoplasmic antibodies and disease activity during long-term follow-up of 70 patients with systemic vasculitis.

Anti-neutrophil cytoplasmic antibodies (ANCA) have been identified as a diagnostic marker in primary systemic vasculitis, and immunofluorescence assays identify two patterns of binding: cytoplasmic (C-ANCA and perinuclear (P-ANCA). We have examined retrospectively the use of such assays in long-term monitoring of disease activity, in order to determine the relationship between presence of ANCA and relapse, and to assess their suitability as a guide to therapy. Seventy patients were studied over a period of 50 months, using clinical and laboratory criteria for the diagnosis of relapse and the internationally standardised immunofluorescence assay for detection of ANCA. In 19 patients C- or P-ANCA were detectable throughout the study period; six of these (with C-ANCA) relapsed. In 18 patients ANCA were undetectable during long-term follow-up; none of these patients relapsed. In 33 patients C- or P-ANCA were intermittently present; nine of these relapsed and all had C-ANCA detectable at the time of relapse. In six of the nine cases, relapse was accompanied or closely preceded by reappearance of C-ANCA. We conclude that continuing presence and reappearance of ANCA may identify patients who are at risk of relapse and who are most likely to benefit from long-term immunosuppressive therapy.

Antibodies, Antineutrophil Cytoplasmic↗
PubMed1997

Studies of epitope restriction on myeloperoxidase (MPO), an important antigen in systemic vasculitis.

Anti-neutrophil cytoplasmic antibodies are important components of the inflammatory response in patients with systemic vasculitis. Their role in the pathogenesis of these conditions remains incompletely defined. Several antigens have been identified, and MPO is one of the most important. To gain more understanding of the immune mechanisms involved, we were keen to see if the antibody response to MPO was restricted, or whether there was a general loss of tolerance to the whole surface of the molecule. To study the epitopes we employed both ELISA and biosensor technology, and were able to demonstrate restriction both in the number and localization of the epitopes being recognized.

Autoantigens↗
PubMedDate not supplied

Systemic vasculitis associated with Fasciola hepatica infection.

We report the case of a 50-year-old man who presented with systemic vasculitis associated with Fasciola hepatica infection. The patient presented with severe skin, kidney, spleen, ophthalmic, and neurological compromise. An immunological examination for primary vasculitis was negative and other infections were discounted by microbiological and serological analyses. The patient was treated with steroids without clinical response. The Fasciola hepatica infection was confirmed by the presence of specific immunoglobulin G (IgG) serum antibodies detected by a quantitative enzyme-linked immunosorbent assay (ELISA) with an optical density (OD) of 0.483 OD units (normal value<0.170 OD units) and a high-titre complement fixation (1/80 dilution). The patient received treatment with triclabendazole and all symptoms and systemic manifestations resolved within weeks. Hence, this previously unreported vasculitis-associated infection, if identified opportunely, can be treated and cured.

Animals↗
PubMed1995

Treatment of anti-neutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis with high-dose intravenous immunoglobulin.

In this uncontrolled study 15 patients with ANCA-associated systemic vasculitis, who were poor responders to conventional therapy, were treated with single or multiple courses of intravenous immunoglobulin (IVIG), 30 g/day over 5 days. Clinical and serological evaluation was performed before and 4 weeks after IVIG. Six of the 15 patients experienced clinically significant benefit from IVIG. Improvement was confined to single organ manifestations (skin, ENT findings), no improvement was seen with conjunctivitis and scleritis, pericarditis or nephritis. No patient experienced complete remission after IVIG. Repeated courses of IVIG at 4-week intervals were no more effective than single courses. In six anti-proteinase 3 (PR3)-positive patients pretreatment sera were incubated with F(ab')2 fragments of the IVIG preparation in vitro to measure the inhibitory effect of IVIG on anti-PR3 activity. An inhibition of anti-PR3 activity by 25-70% was observed; this did not correlate with clinical effects. Approximately 40% of patients benefited from IVIG treatment, though complete remission of disease activity did not occur. Neither clinical characteristics nor the inhibitory effect of the IVIG preparation on serum anti-PR3 activity in vitro predicted clinical response to this treatment modality.

Adult↗
PubMed2005

Angiography-negative primary central nervous system vasculitis in children: a newly recognized inflammatory central nervous system disease.

Inflammatory central nervous system (CNS) diseases in childhood comprise a wide spectrum of heterogeneous conditions. We studied 4 children with primary CNS vasculitis in whom results of magnetic resonance imaging studies were abnormal but results of conventional angiography were normal. We determined that angiography-negative, biopsy-confirmed primary small-vessel CNS vasculitis is a previously unrecognized distinct disease entity in children. The diagnosis must be considered in a child with a progressive, acquired diffuse or focal neurologic deficit, even if the results of conventional angiography are normal. A lesional brain biopsy is required to confirm the diagnosis. Use of immunosuppressive therapy plus aspirin leads to an excellent neurologic outcome.

Adolescent↗
PubMed2004

Inflammation and arterial stiffness in systemic vasculitis: a model of vascular inflammation.

OBJECTIVE: Arterial stiffness, an independent determinant of cardiovascular risk, is regulated by both structural and functional factors, including endothelium-derived nitric oxide. Endothelial dysfunction is associated with acute and chronic systemic inflammation. However, the role of systemic inflammation in arterial stiffening has not been determined. The aim of this study was to investigate the relationship between inflammation and arterial stiffness in patients with antineutrophil cytoplasmic antibody-associated systemic vasculitis (AASV) as a model of systemic inflammation. METHODS: Thirty-one patients with AASV (15 with active disease) and 32 age-matched controls were studied. Pulse wave velocity (PWV) and the augmentation index (AIx) were assessed noninvasively and related to serum levels of C-reactive protein (CRP), interleukin-6, and tumor necrosis factor alpha. RESULTS: In subjects with active disease, the AIx, PWV, and level of CRP were elevated compared with that in controls (mean +/- SEM 31 +/- 3% versus 22 +/- 2% [P = 0.003], 9.2 +/- 0.7 versus 7.5 +/- 0.3 meters/second [P = 0.03], and 16.0 +/- 4.0 versus 1.1 +/- 0.1 mg/liter [P < 0.001], respectively). However, PWV and the AIx were not significantly different between patients with disease in remission and controls (8.0 +/- 0.5 versus 7.5 +/- 0.3 meters/second and 19 +/- 3% versus 22 +/- 2%, respectively). The CRP level was positively correlated with both PWV and the AIx. Multiple regression analysis indicated that age, mean arterial pressure (MAP), and CRP were independently related to PWV, and that age, MAP, CRP, sex, and heart rate were associated with the AIx. CONCLUSION: These data indicate that AASV is associated with increased arterial stiffness, and that stiffness correlates with the degree of active inflammation.

Adolescent↗
PubMed1993

Alpha-1-proteinase inhibitor and pulmonary haemorrhage in systemic vasculitis.

Alpha-1-proteinase inhibitor phenotypes and levels were examined in 40 antineutrophil cytoplasmic antibody positive cases of systemic vasculitis. An excess of PiZ and PiS alleles were associated with the development of pulmonary haemorrhage and alpha-1-proteinase inhibitor levels were lower in the subgroup with pulmonary haemorrhage. However, this allelic imbalance and reduced alpha-1-proteinase inhibitor level was not confined to antiproteinase 3 positive patients and did not appear to be associated with other organ involvement or disease severity.

Alleles↗