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Hoffmann, R. A wiki for the life sciences where authorship matters. Nature Genetics (2008)
MeSH Review


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Disease relevance of Vasculitis


High impact information on Vasculitis


Chemical compound and disease context of Vasculitis


Biological context of Vasculitis


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


  1. Anti-neutrophil cytoplasmic autoantibodies with specificity for myeloperoxidase in patients with systemic vasculitis and idiopathic necrotizing and crescentic glomerulonephritis. Falk, R.J., Jennette, J.C. N. Engl. J. Med. (1988) [Pubmed]
  2. Elevated platelet-associated IgG in thrombotic thrombocytopenic purpura. Morrison, J., McMillan, R. JAMA (1977) [Pubmed]
  3. The role of B cells in lpr/lpr-induced autoimmunity. Shlomchik, M.J., Madaio, M.P., Ni, D., Trounstein, M., Huszar, D. J. Exp. Med. (1994) [Pubmed]
  4. Defect of a complement receptor 3 epitope in a patient with systemic lupus erythematosus. Witte, T., Dumoulin, F.L., Gessner, J.E., Schubert, J., Götze, O., Neumann, C., Todd, R.F., Deicher, H., Schmidt, R.E. J. Clin. Invest. (1993) [Pubmed]
  5. The Raji cell radioimmune assay for detecting immune complexes in human sera. Theofilopoulos, A.N., Wilson, C.B., Dixon, F.J. J. Clin. Invest. (1976) [Pubmed]
  6. Ciprofloxacin-induced vasculitis. Choe, U., Rothschild, B.M., Laitman, L. N. Engl. J. Med. (1989) [Pubmed]
  7. Treatment of vasculitis with cyclophosphamide. Elkon, K.B., Hughes, G.R., Pussell, B.A. N. Engl. J. Med. (1979) [Pubmed]
  8. Phenformin in the treatment of livedo vasculitis. Basler, R.S., Jones, H.E. N. Engl. J. Med. (1978) [Pubmed]
  9. Murine gamma-herpesvirus 68 causes severe large-vessel arteritis in mice lacking interferon-gamma responsiveness: a new model for virus-induced vascular disease. Weck, K.E., Dal Canto, A.J., Gould, J.D., O'Guin, A.K., Roth, K.A., Saffitz, J.E., Speck, S.H., Virgin, H.W. Nat. Med. (1997) [Pubmed]
  10. Endothelin-1 and vasculitis. Kanno, K., Hirata, Y., Numano, F., Emori, T., Ohta, K., Shichiri, M., Marumo, F. JAMA (1990) [Pubmed]
  11. Propylthiouracil-induced cutaneous vasculitis. Case presentation and review of the literature. Vasily, D.B., Tyler, W.B. JAMA (1980) [Pubmed]
  12. Leukoclastic vasculitis from cimetidine. Dernbach, W.K., Taylor, G. JAMA (1981) [Pubmed]
  13. Vasculitis and melphalan. Skehan, M.W., Bernath, A.M. JAMA (1978) [Pubmed]
  14. Cryoimmunoglobulinemia in rheumatoid arthritis. Significance in serum of patients with rheumatoid vasculitis. Weisman, M., Zvaifler, N. J. Clin. Invest. (1975) [Pubmed]
  15. Allogeneic hematolymphoid microchimerism and prevention of autoimmune disease in the rat. A relationship between allo- and autoimmunity. Delaney, C.P., Murase, N., Chen-Woan, M., Fung, J.J., Starzl, T.E., Demetris, A.J. J. Clin. Invest. (1996) [Pubmed]
  16. Upregulated expression and function of integrin adhesive receptors in systemic lupus erythematosus patients with vasculitis. Takeuchi, T., Amano, K., Sekine, H., Koide, J., Abe, T. J. Clin. Invest. (1993) [Pubmed]
  17. Particular HLA-DRB1 shared epitope genotypes are strongly associated with rheumatoid vasculitis. Gorman, J.D., David-Vaudey, E., Pai, M., Lum, R.F., Criswell, L.A. Arthritis Rheum. (2004) [Pubmed]
  18. Fc receptor-independent development of autoimmune glomerulonephritis in lupus-prone MRL/lpr mice. Matsumoto, K., Watanabe, N., Akikusa, B., Kurasawa, K., Matsumura, R., Saito, Y., Iwamoto, I., Saito, T. Arthritis Rheum. (2003) [Pubmed]
  19. Coagulation abnormalities in rheumatoid disease. Conn, D.L., McDuffie, F.C., Kazmier, F.J., Schroeter, A.L., Sun, N.C. Arthritis Rheum. (1976) [Pubmed]
  20. Fatal postpartum vasculitis in systemic lupus erythematosus. Hubscher, O., Eimon, A., Arana, R.M. Arthritis Rheum. (1996) [Pubmed]
  21. Wegener's granulomatosis: anti-proteinase 3 antibodies are potent inductors of human endothelial cell signaling and leakage response. Sibelius, U., Hattar, K., Schenkel, A., Noll, T., Csernok, E., Gross, W.L., Mayet, W.J., Piper, H.M., Seeger, W., Grimminger, F. J. Exp. Med. (1998) [Pubmed]
  22. Antineutrophil cytoplasmic antibodies induce monocyte IL-8 release. Role of surface proteinase-3, alpha1-antitrypsin, and Fcgamma receptors. Ralston, D.R., Marsh, C.B., Lowe, M.P., Wewers, M.D. J. Clin. Invest. (1997) [Pubmed]
  23. Systemic lupus erythematosus and intestinal venulitis. Weiser, M.M., Andres, G.A., Brentjens, J.R., Evans, J.T., Reichlin, M. Gastroenterology (1981) [Pubmed]
  24. Factor VIII von Willebrand protein in haemolytic uraemic syndrome and systemic vasculitides. Rose, P.E., Struthers, G.S., Robertson, M., Kavi, J., Chant, I., Taylor, C.M. Lancet (1990) [Pubmed]
  25. Major histocompatibility complex class I-recognizing receptors are disease risk genes in rheumatoid arthritis. Yen, J.H., Moore, B.E., Nakajima, T., Scholl, D., Schaid, D.J., Weyand, C.M., Goronzy, J.J. J. Exp. Med. (2001) [Pubmed]
  26. Effect of tumor necrosis factor-induced integrin activation on Fc gamma receptor II-mediated signal transduction: relevance for activation of neutrophils by anti-proteinase 3 or anti-myeloperoxidase antibodies. Reumaux, D., Vossebeld, P.J., Roos, D., Verhoeven, A.J. Blood (1995) [Pubmed]
  27. Myeloid-related proteins 8 and 14 induce a specific inflammatory response in human microvascular endothelial cells. Viemann, D., Strey, A., Janning, A., Jurk, K., Klimmek, K., Vogl, T., Hirono, K., Ichida, F., Foell, D., Kehrel, B., Gerke, V., Sorg, C., Roth, J. Blood (2005) [Pubmed]
  28. T lymphocyte adhesion mechanisms within inflamed human kidney: studies with a Stamper-Woodruff assay. Chakravorty, S.J., Howie, A.J., Cockwell, P., Adu, D., Savage, C.O. Am. J. Pathol. (1999) [Pubmed]
  29. A vasculopathy with deposition of lambda light chain crystals. Stone, G.C., Wall, B.A., Oppliger, I.R., Wener, M.H., Jolly, S.L., Aguirre, A., Dwyer, R., Simkin, P.A. Ann. Intern. Med. (1989) [Pubmed]
  30. The spectrum of vasculitis: clinical, pathologic, immunologic and therapeutic considerations. Fauci, A.S., Haynes, B., Katz, P. Ann. Intern. Med. (1978) [Pubmed]
  31. Prevention of infection due to Pneumocystis spp. in human immunodeficiency virus-negative immunocompromised patients. Rodriguez, M., Fishman, J.A. Clin. Microbiol. Rev. (2004) [Pubmed]
  32. Atypical antineutrophil cytoplasmic antibodies with perinuclear fluorescence in chronic inflammatory bowel diseases and hepatobiliary disorders colocalize with nuclear lamina proteins. Terjung, B., Herzog, V., Worman, H.J., Gestmann, I., Bauer, C., Sauerbruch, T., Spengler, U. Hepatology (1998) [Pubmed]
  33. Antineutrophil cytoplasmic antibodies reacting with the pro form of proteinase 3 and disease activity in patients with Wegener's granulomatosis and microscopic polyangiitis. Russell, K.A., Fass, D.N., Specks, U. Arthritis Rheum. (2001) [Pubmed]
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