Disease relevance of Vasculitis

• Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis [1].
• Analysis of the patient's platelets for immunoglobulins showed abnormally elevated levels in the range of those seen in thrombocytopenic patients with systemic lupus erythematosus, immune vasculitis, and infectious mononucleosis [2].
• In contrast, lpr/lpr littermates that had B cells had severe nephritis and vasculitis, as well as autoantibodies [3].
• Clinically this patient exhibited predominantly cutaneous manifestations of a systemic lupus erythematosus with an immune vasculitis and panniculitis [4].
• Thereby immune complexes were detected and quantitated in serum hepatitis, systemic lupus erythematosus, vasculitis, subacute sclerosing panencephalitis, dengue hemorrhagic fever, and malignancies [5].

High impact information on Vasculitis

• Ciprofloxacin-induced vasculitis [6].
• Treatment of vasculitis with cyclophosphamide [7].
• Phenformin in the treatment of livedo vasculitis [8].
• These studies demonstrate that IFN-gamma is essential for control of chronic vascular pathology induced by gammaHV68 and suggest gamma-herpesviruses as candidate etiologic agents for human vasculitis [9].
• Endothelin-1 and vasculitis [10].

Chemical compound and disease context of Vasculitis

• Propylthiouracil-induced cutaneous vasculitis. Case presentation and review of the literature [11].
• Leukoclastic vasculitis from cimetidine [12].
• Vasculitis and melphalan [13].
• Serum antiglobulin titers were higher, and serum C3 levels were lower, in vasculitis patients compared to rheumatoid patients without vasclitis [14].
• Administration of HgCl2 to naive BN rats induced marked autoantibody production, systemic vasculitis and lymphocytic infiltration of the kidneys, liver and skin in all of the animals and a 47% mortality [15].

Biological context of Vasculitis

• These results introduce a possibility that upregulation of integrin adhesive receptors has a potential role in the pathogenesis of vasculitis in SLE [16].
• Particular HLA-DRB1 shared epitope genotypes are strongly associated with rheumatoid vasculitis [17].
• OBJECTIVE: To determine the role of Fc receptors (FcR), which play crucial roles in antibody and immune complex-mediated inflammation and autoimmunity, including glomerulonephritis (GN), in the development of autoimmune GN and vasculitis in MRL/lpr mice, one of the most widely used lupus-prone mouse models [18].
• The results suggest overcompensated ICF occurring in rheumatoid arthritis, but rheumatoid patients with vasculitis cannot be clearly distinguished from those without vasculitis on the basis of the usual tests performed for coagulation and fibrinolysis abnormalities [19].
• Fatal postpartum vasculitis in systemic lupus erythematosus [20].

Anatomical context of Vasculitis

• Associated metabolic events and the loss of endothelial barrier properties suggest that anti-PR3-induced activation of endothelial cells may contribute to the pathogenetic sequelae of autoimmune vasculitis characterizing WG [21].
• Cytoplasmic antineutrophil cytoplasmic antibodies (cANCA) that accompany the neutrophilic vasculitis seen in Wegener's granulomatosis (WG), are directed against proteinase-3 (PR-3), a serine proteinase which is located in azurophilic granules of neutrophils and monocytes [22].
• This report documents that in systemic lupus erythematosus the intestine can be affected with a vasculitis similar to that seen in the skin and can cause thickening of the basement membrane of intestinal villi [23].
• Plasma samples from normal subjects, children with haemolytic uraemic syndrome (HUS), and adults with vasculitis and vWF prepared from endothelium were treated with DTT before vWF assay. vWF in HUS and vasculitis resembled the endothelial form in being resistant to reduction [24].
• A full-thickness section of jejunum obtained at laparotomy demonstrated a severe, diffuse vasculitis involving the venules of the submucosa and muscularis externa with infiltration of polymorphonuclear leukocytes and macrophages, and deposits of C3 [23].

Gene context of Vasculitis

• The KIR2DS2 gene was significantly enriched among patients with rheumatoid vasculitis compared with normal individuals (odds ratio 5.56, P = 0.001) and patients with RA but no vasculitis (odds ratio 7.96, P = 0.001) [25].
• To test the hypothesis that the KIR2DS2 gene is involved in the development of vasculitis, association studies were performed [25].
• The two main targets of ANCA in vasculitis are proteinase 3 (PR3) and myeloperoxidase (MPO) [26].
• We demonstrated that the expression of MRP8/MRP14 closely correlated with the inflammatory activity in systemic vasculitis, confirming the important role of these proteins for distinct inflammatory reactions in endothelia [27].
• Glomerular adhesion in vasculitic GN and tubulointerstitial adhesion in acute rejection were particularly sensitive to both anti-ICAM-1 and anti-VCAM-1 antibodies, indicating a prominent role for ICAM-1 and VCAM-1 at glomerular sites in vasculitis and at tubulointerstitial sites in rejection [28].

Analytical, diagnostic and therapeutic context of Vasculitis

• Although the presentation of these patients resembled that of systemic necrotizing vasculitis, histologic evidence of inflammation was lacking and their subsequent rapid clinical deterioration was not altered by corticosteroid therapy, and in one case cyclophosphamide and plasmapheresis [29].
• In recent years, several of the more serious vasculitides, such as Wegener's granulomatosis and the systemic necrotizing vasculitides of the polyarteritis nodosa group, which formerly had extremely poor prognoses, have been shown to be extraordinarily responsive to chronic low-dose cytotoxic therapy, particularly cyclophosphamide [30].
• Populations at risk also include patients with hematologic and nonhematologic malignancies, hematopoietic stem cell transplant recipients, solid-organ recipients, and patients receiving immunosuppressive therapies for connective tissue disorders and vasculitides [31].
• To further investigate this hypothesis, we examined sera from patients with ulcerative colitis, primary sclerosing cholangitis, autoimmune hepatitis or systemic vasculitis on ethanol or formaldehyde-fixed neutrophils using confocal laser scanning microscopy and immunoelectron microscopy [32].
• METHODS: Sera from 61 PR3 ANCA-positive patients with WG or MPA were assayed by capture enzyme-linked immunosorbent assay using pro-rPR3 and rPR3 as target antigens, and were correlated with disease activity as determined by the Birmingham Vasculitis Activity Score (BVAS) [33].

References