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Results for “Systemic Vasculitis”

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At least 505 records · Page 28Linked to original sources
PubMed1990

Antibodies directed against neutrophils (C-ANCA and P-ANCA) are of distinct diagnostic value in systemic vasculitis.

In a prospective study, sera from over 700 patients with suspected vasculitis, including over 200 patients undergoing renal biopsy, were examined for antibodies to neutrophil cytoplasmic antigen (ANCA). An indirect immunofluorescence assay on ethanol fixed human neutrophils identified two types of autoantibody: C-ANCA, which produces diffuse cytoplasmic staining and P-ANCA, which produces an artefactual nuclear/perinuclear staining pattern. The diagnosis of patients in whom ANCA of either form was found was established following case note review according to defined diagnostic criteria. Forty of 45 patients whose sera contained C-ANCA at a titre of greater than or equal to 1/40 satisfied the diagnostic criteria for Wegener's granulomatosis or microscopic polyarteritis. Twelve of 30 patients with P-ANCA at a titre of greater than or equal to 1/40 were also classified as having one of these disorders. Seventeen of the remaining 18 patients had immune-mediated disorders with deep organ damage. Of 47 ANCA-positive patients who underwent renal biopsy, 21 had glomerulonephritis associated with Wegener's glomerulonephritis and a further 22 had a necrotizing or crescentic glomerulonephritis. Ethanol fixation is important for discrimination of C-ANCA and P-ANCA. C-ANCA are highly specific for Wegener's granulomatosis and microscopic polyarteritis. In patients undergoing renal biopsy, the presence of these antibodies is highly specific for a necrotizing or crescentic glomerulonephritis.

Arteritis↗
PubMed1996

Intravenous immunoglobulin as sole therapy for systemic vasculitis.

High-dose, pooled, i.v. immunoglobulin (IVIg) is a potential, alternative treatment for Wegener's granulomatosis (WG) and microscopic polyangiitis (MPA) which has shown promise in the treatment of refractory disease when administered with continuing immunosuppression. This study of six new patients with antineutrophil cytoplasmic antibody (ANCA)-positive vasculitis and early disease, without threatened vital organ function, examined the therapeutic response to treatment with IVIg alone. IVIg was well tolerated and all six patients had early reductions in disease activity. Four entered full, clinical remission which lasted for at least 1 yr, while in two the responses were partial and transient, and they subsequently required conventional treatment. After 16-48 months of follow-up, two of the four patients in full remission relapsed, but the other two have remained well.

Adult↗
PubMed1996

New developments in the pathogenesis of systemic vasculitis.

Over the past few years, novel pathogenic mechanisms leading to vascular inflammation and injury have been discovered. Circumstantial evidence that supports a pathogenic role for antineutrophil cytoplasmic antibodies (ANCA) in mediating vessel damage includes in vitro studies showing that ANCA promote neutrophil activation and endothelial injury. In addition, several animal models of ANCA-induced vasculitis and glomerulonephritis have been developed. The role of T cell-mediated immune mechanisms in vasculitis has been strengthened by molecular studies demonstrating clonal expansion of selected T cell populations in inflammatory foci, possibly recognizing a disease-relevant antigen. There is also increasing awareness of the important role that adhesion molecules may play in the development of vascular inflammatory infiltrates. The putative participation of cytokines and growth factors, released by inflammatory cells, in vessel occlusion and organ dysfunction, is a focus of increasing research activity.

Animals↗
PubMed1998

Infections and systemic vasculitis.

Bacterial infections can clinically mimic a primary vasculitic syndrome and can directly cause a vasculitis that may respond to prompt recognition and treatment of the infection. Increasing direct and indirect evidence is establishing links between certain infections and vasculopathies, including aortitis, atherosclerosis, and Wegener's granulomatosis. The literature on the hepatitis viruses, particularly hepatitis C, and various vasculitic syndromes continues to grow. The spectrum of clinical syndromes, including "essential" cryoglobulinemia (elicited by the hepatitis viruses) and HIV, continues to broaden. Clinical trials have begun to demonstrate the therapeutic value of antiviral therapy in patients with these conditions.

Bacterial Infections↗
PubMed1999

New concepts in treatment protocols for severe systemic vasculitis.

Glucocorticosteroids (GCs) are the drug of choice in all clinical types of giant cell arteritis (GCA); a study delineated that an unexpectedly high percentage of patients required long-term GCs, with the consequence of significant complications attributable to GC therapy. Azathioprine and methotrexate are recommended as GC-sparing drugs. Cyclosporin A was found to confer no additive effect versus GC treatment alone. Depot GCs intramuscularly every 3 weeks decreased the cumulative GC dose and were associated with fewer bone fractions compared with daily oral GCs. Pulse cyclophosphamide has been shown to be as effective as the standard therapy in necrotizing vasculitides; however, an alarmingly high rate of infections was observed in this study in both arms possibly related to the high dosage of GCs. New drugs such as mycophenolate mofetil and leflunomide appear as alternatives as maintenance therapy in antineutrophil cytoplasm autoantibody-associated vasculitides in pilot studies. Interferon-alpha (IFN-alpha) has been shown to be effective in treatment-resistant Churg-Strauss syndrome, and IFN-alpha or ribavirin can be used successfully in essential mixed cryoglobulinemia (induced by hepatitis C virus). Thalidomide was shown to be effective for treating oral and genital ulcers and follicular lesions in Behçet's syndrome; severe refractory Behçet's syndrome uveitis responded to treatment with IFN-alpha.

Cyclophosphamide↗