Pathogenesis of Wegener's granulomatosis: Current concepts

Expert Reviews in Molecular Medicine

Volumn 7, Issue 8, 2005, Pages

  Sarraf, Pasha ^a   Sneller, Michael C ^a  

^a NATIONAL INSTITUTE OF ALLERGY AND INFECTIOUS DISEASES   (United States)

Author keywords

ANCA; Antineutrophil cytoplasmic antibody; Autoimmune; Glomerulonephritis; Granuloma; Myeloperoxidase; Proteinase 3; Vasculitis; Wegener's granulomatosis

Indexed keywords

BIOLOGICAL MARKER; CYCLOPHOSPHAMIDE; ETANERCEPT; GLUCOCORTICOID; INFLIXIMAB; NEUTROPHIL CYTOPLASMIC ANTIBODY; RITUXIMAB; AUTOANTIGEN; CHEMOKINE; CYTOKINE; MYELOBLASTIN; PEROXIDASE;

ANTIBODY DETECTION; CLINICAL TRIAL; FATALITY; GLOMERULONEPHRITIS; GRANULOMATOUS INFLAMMATION; HUMAN; LUNG GRANULOMATOSIS; MICROANGIOPATHY; NECROSIS; NONHUMAN; PATHOGENESIS; PRIORITY JOURNAL; PROTEIN FUNCTION; RELAPSE; REMISSION; REVIEW; WEGENER GRANULOMATOSIS; ADOLESCENT; ADULT; ANIMAL; AUTOIMMUNE DISEASE; CHILD; FEMALE; GENETICS; IMMUNOLOGY; KIDNEY; LUNG; MALE; MOUSE; MOUSE MUTANT; PATHOLOGY; PHYSIOLOGY; TH1 CELL;

ANIMALIA;

ADOLESCENT; ADULT; ANIMALS; ANTIBODIES, ANTINEUTROPHIL CYTOPLASMIC; AUTOANTIGENS; AUTOIMMUNE DISEASES; CHEMOKINES; CHILD; CYTOKINES; FEMALE; HUMANS; KIDNEY; LUNG; MALE; MICE; MICE, KNOCKOUT; MYELOBLASTIN; PEROXIDASE; TH1 CELLS; WEGENER GRANULOMATOSIS;

EID: 31044449754     PISSN: 14623994     EISSN: None     Source Type: Journal    
DOI: 10.1017/S146239940500921X     Document Type: Review

References (94)

1. Hoffman, G.S. et al. (1992) Wegener granulomatosis: an analysis of 158 patients. Ann Intern Med 116, 488-498,
2. Reinhold-Keller, E. et al. (2000) An interdisciplinary approach to the care of patients with Wegener's granulomatosis: long-term outcome in 155 patients. Arthritis Rheum 43, 1021-1032,
3. Gonzalez-Gay, M.A. and Garcia-Porrua, C. (2001) Epidemiology of the vasculitides. Rheum Dis Clin North Am 27, 729-749,
4. Koldingsnes, W. and Nossent, H. (2000) Epidemiology of Wegener's granulomatosis in northern Norway. Arthritis Rheum 43, 2481-2487,
5. Raynauld, J.P., Bloch, D.A. and Fries, J.F. (1993) Seasonal variation in the onset of Wegener's granulomatosis, polyarteritis nodosa and giant cell arteritis. J Rheumatol 20, 1524-1526,
6. Albert, D. et al. (2004) Wegener's granulomatosis: Possible role of environmental agents in its pathogenesis. Arthritis Rheum 51, 656-664,
7. Lane, S.E. et al. (2003) Are environmental factors important in primary systemic vasculitis? A case-control study. Arthritis Rheum 48, 814-823,
8. Abdou, N.I. et al. (2002) Wegener's granulomatosis: survey of 701 patients in North America. Changes in outcome in the 1990s. J Rheumatol 29, 309-316,
9. Walton, E.W. (1958) Giant-cell granuloma of the respiratory tract (Wegener's granulomatosis). Br Med J 34, 265-270,
10. Fahey, J.L. et al. (1954) Wegener's granulomatosis. Am J Med 17, 168-179,
11. Travis, W.D. et al. (1991) Surgical pathology of the lung in Wegener's granulomatosis. Review of 87 open lung biopsies from 67 patients. Am J Surg Pathol 15, 315-333,
12. Godman, G.C. and Churg, J. (1954) Wegener's granulomatosis: pathology and review of the literature. AMA Arch Pathol 58, 533-553,
13. Horn, R.G. et al. (1974) Renal biopsy pathology in Wegener's granulomatosis. Am J Pathol 74, 423-440,
14. Langford, C.A. and Sneller, M.C. (2001) Update on the diagnosis and treatment of Wegener's granulomatosis. Adv Intern Med 46, 177-206,
15. Langford, C.A. (2003) Treatment of ANCA-associated vasculitis. N Engl J Med 349, 3-4,
16. Davies, D.J. et al. (1982) Segmental necrotising glomerulonephritis with antineutrophil antibody: possible arbovirus aetiology? Br Med J (Clin Res Ed) 285, 606,
17. Hall, J.B. et al. (1984) Vasculitis and glomerulonephritis: a subgroup with an antineutrophil cytoplasmic antibody. Aust N Z J Med 14, 277-278,
18. van der Woude, F.J. et al. (1985) Autoantibodies against neutrophils and monocytes: tool for diagnosis and marker of disease activity in Wegener's granulomatosis. Lancet 1, 425-429,
19. Tervaert, J.W. et al. (1990) Prevention of relapses in Wegener's granulomatosis by treatment based on antineutrophil cytoplasmic antibody titre. Lancet 336, 709-711,
20. Nolle, B. et al. (1989) Anticytoplasmic autoantibodies: their immunodiagnostic value in Wegener granulomatosis. Ann Intern Med 111, 28-40,
21. Hoffman, G.S. and Specks, U. (1998) Antineutrophil cytoplasmic antibodies. Arthritis Rheum 41, 1521-1537,
22. Boomsma, M.M. et al. (2000) Prediction of relapses in Wegener's granulomatosis by measurement of antineutrophil cytoplasmic antibody levels: a prospective study. Arthritis Rheum 43, 2025-2033,
23. van der Geld, Y.M., Limburg, P.C. and Kallenberg, C.G. (2001) Proteinase 3, Wegener's autoantigen: from gene to antigen. J Leukoc Biol 69, 177-190,
24. Halbwachs-Mecarelli, L. et al. (1995) Bimodal distribution of proteinase 3 (PR3) surface expression reflects a constitutive heterogeneity in the polymorphonuclear neutrophil pool. FEBS Lett 374, 29-33,
25. Csernok, E. et al. (1994) Activated neutrophils express proteinase 3 on their plasma membrane in vitro and in vivo. Clin Exp Immunol 95, 244-250,
26. David, A. et al. (2003) Interaction of proteinase 3 with CD11b/CD18 (beta2 integrin) on the cell membrane of human neutrophils. J Leukoc Biol 74, 551-557,
27. Mayet, W.J. et al. (1993) Human endothelial cells express proteinase 3, the target antigen of anticytoplasmic antibodies in Wegener's granulomatosis. Blood 82, 1221-1229,
28. Pezzato, E. et al. (2003) Proteinase-3 directly activates MMP-2 and degrades gelatin and Matrigel; differential inhibition by (-)epigallocatechin-3-gallate. J Leukoc Biol 74, 88-94,
29. Ballieux, B.E. et al. (1994) Binding of proteinase 3 and myeloperoxidase to endothelial cells: ANCA-mediated endothelial damage through ADCC? Clin Exp Immunol 97, 52-60,
30. Robache-Gallea, S. et al. (1995) In vitro processing of human tumor necrosis factor-alpha. J Biol Chem 270, 23688-23692,
31. Leid, R.W. et al. (1993) Cleavage and inactivation of human C1 inhibitor by the human leukocyte proteinase, proteinase 3. Eur J Immunol 23, 2939-2944,
32. Dolman, K.M. et al. (1992) Determination of proteinase 3-alpha 1-antitrypsin complexes in inflammatory fluids. FEBS Lett 314, 117-121,
33. Fang, F.C. (2004) Antimicrobial reactive oxygen and nitrogen species: concepts and controversies. Nat Rev Microbiol 2, 820-832,
34. Baldus, S. et al. (2001) Endothelial transcytosis of myeloperoxidase confers specificity to vascular ECM proteins as targets of tyrosine nitration. J Clin Invest 108, 1759-1770,
35. Aratani, Y. et al. (1999) Severe impairment in early host defense against Candida albicans in mice deficient in myeloperoxidase. Infect Immun 67, 1828-1836,
36. Falk, R.J. et al. (1990) Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro. Proc Natl Acad Sci U S A 87, 4115-4119,
37. Porges, A.J. et al. (1994) Anti-neutrophil cytoplasmic antibodies engage and activate human neutrophils via Fc gamma RIIa. J Immunol 153, 1271-1280,
38. Savage, C.O. et al. (1992) Autoantibodies developing to myeloperoxidase and proteinase 3 in systemic vasculitis stimulate neutrophil cytotoxicity toward cultured endothelial cells. Am J Pathol 141, 335-342,
39. Brooks, C.J. et al. (1996) IL-1 beta production by human polymorphonuclear leucocytes stimulated by anti-neutrophil cytoplasmic autoantibodies: relevance to systemic vasculitis. Clin Exp Immunol. 106, 273-279,
40. Mayet, W. et al. (1999) Anti-PR-3 antibodies induce endothelial IL-8 release. Eur J Clin Invest 29, 973-979,
41. Grimminger, F. et al. (1996) Neutrophil activation by anti-proteinase 3 antibodies in Wegener's granulomatosis: role of exogenous arachidonic acid and leukotriene B4 generation. J Exp Med 184, 1567-1572,
42. Brouwer, E. et al. (1993) Antimyeloperoxidase-associated proliferative glomerulonephritis: an animal model. J Exp Med 177, 905-914,
43. Heeringa, P. et al. (1996) Autoantibodies to myeloperoxidase aggravate mild anti-glomerular-basement-membrane-mediated glomerular injury in the rat. Am J Pathol 149, 1695-1706,
44. Xiao, H. et al. (2002) Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. J Clin Invest 110, 955-963,
45. Falk, R.J. and Jennette, J.C. (2004) Thoughts about the classification of small vessel vasculitis. J Nephrol 17 Suppl 8, S3-9,
46. Pfister, H. et al. (2004) Antineutrophil cytoplasmic autoantibodies against the murine homolog of proteinase 3 (Wegener autoantigen) are pathogenic in vivo. Blood 104, 1411-1418,
47. Ravetch, J.V. (2002) A full complement of receptors in immune complex diseases. J Clin Invest 110, 1759-1761,
48. Abdel-Salam, B. et al. (2004) Autoantibodies to neutrophil cytoplasmic antigens (ANCA) do not bind to polymorphonuclear neutrophils in blood. Kidney Int 66, 1009-1017,
49. Heeringa, P. and Tervaert, J.W. (2004) Pathophysiology of ANCA-associated vasculitides: are ANCA really pathogenic? Kidney Int 65, 1564-1567,
50. Kerr, G.S. et al. (1993) Limited prognostic value of changes in antineutrophil cytoplasmic antibody titer in patients with Wegener's granulomatosis. Arthritis Rheum 36, 365-371,
51. James, D.G. (2000) A clinicopathological classification of granulomatous disorders. Postgrad Med J 76, 457-465,
52. Sneller, M.C. (2002) Granuloma formation, implications for the pathogenesis of vasculitis. Cleve Clin J Med 69 Suppl 2, SII40-43,
53. D'Souza, C.D. et al. (1997) An anti-inflammatory role for gamma delta T lymphocytes in acquired immunity to Mycobacterium tuberculosis. J Immunol 158, 1217-1221,
54. Hess, J. et al. (2000) Exploiting the immune system: toward new vaccines against intracellular bacteria. Adv Immunol 75, 1-88,
55. Adams, D.O. (1974) The structure of mononuclear phagocytes differentiating in vivo. I. Sequential fine and histologic studies of the effect of Bacillus Calmette-Guerin (BCG). Am J Pathol 76, 17-48,
56. Seiler, P. et al. (2003) Early granuloma formation after aerosol Mycobacterium tuberculosis infection is regulated by neutrophils via CXCR3-signaling chemokines. Eur J Immunol 33, 2676-2686,
57. Gonzalez-Juarrero, M. et al. (2001) Temporal and spatial arrangement of lymphocytes within lung granulomas induced by aerosol infection with Mycobacterium tuberculosis. Infect Immun 69, 1722-1728,
58. Adams, D.O. (1976) The granulomatous inflammatory response. A review. Am J Pathol 84, 164-191,
59. Bosio, C.M., Gardner, D. and Elkins, K.L. (2000) Infection of B cell-deficient mice with CDC 1551, a clinical isolate of Mycobacterium tuberculosis: delay in dissemination and development of lung pathology. J Immunol 164, 6417-6425,
60. Co, D.O. et al. (2004) Mycobacterial granulomas: keys to a long-lasting host-pathogen relationship. Clin Immunol 113, 130-136,
61. Caruso, A.M. et al. (1999) Mice deficient in CD4 T cells have only transiently diminished levels of IFN-gamma, yet succumb to tuberculosis. J Immunol 162, 5407-5416,
62. Saunders, B.M. et al. (2002) CD4 is required for the development of a protective granulomatous response to pulmonary tuberculosis. Cell Immunol 216, 65-72,
63. Horsburgh, C.R., Jr. (1991) Mycobacterium avium complex infection in the acquired immunodeficiency syndrome. N Engl J Med 324, 1332-1338,
64. Di Perri, G. et al. (1996) Comparative histopathological study of pulmonary tuberculosis in human immunodeficiency virus-infected and non-infected patients. Tuber Lung Dis 77, 244-249,
65. Kunkel, S.L. et al. (1996) Th1 and Th2 responses regulate experimental lung granuloma development. Sarcoidosis Vasc Diffuse Lung Dis 13, 120-128,
66. Cooper, A.M. et al. (1993) Disseminated tuberculosis in interferon gamma gene-disrupted mice. J Exp Med 178, 2243-2247,
67. Flynn, J.L. et al. (1993) An essential role for interferon gamma in resistance to Mycobacterium tuberculosis infection. J Exp Med 178, 2249-2254,
68. Kamijo, R. et al. (1993) Mice that lack the interferon-gamma receptor have profoundly altered responses to infection with Bacillus Calmette-Guerin and subsequent challenge with lipopolysaccharide. J Exp Med 178, 1435-1440,
69. Watford, W.T. et al. (2003) The biology of IL-12: coordinating innate and adaptive immune responses. Cytokine Growth Factor Rev 14, 361-368,
70. Cooper, A.M. et al. (1997) Interleukin 12 (IL-12) is crucial to the development of protective immunity in mice intravenously infected with mycobacterium tuberculosis. J Exp Med 186, 39-45,
71. Lammas, D.A. et al. (2002) Heterogeneity in the granulomatous response to mycobacterial infection in patients with defined genetic mutations in the interleukin 12-dependent interferon-gamma production pathway. Int J Exp Pathol 83, 1-20,
72. Altare, F. et al. (1998) Impairment of mycobacterial immunity in human interleukin-12 receptor deficiency. Science 280, 1432-1435,
73. de Jong, R. et al. (1998) Severe mycobacterial and Salmonella infections in interleukin-12 receptor-deficient patients. Science 280, 1435-1438,
74. Bazzoni, F. and Beutler, B. (1996) The tumor necrosis factor ligand and receptor families. N Engl J Med 334, 1717-1725,
75. Bean, A.G. et al. (1999) Structural deficiencies in granuloma formation in TNF gene-targeted mice underlie the heightened susceptibility to aerosol Mycobacterium tuberculosis infection, which is not compensated for by lymphotoxin. J Immunol 162, 3504-3511,
76. Marino, M.W. et al. (1997) Characterization of tumor necrosis factor-deficient mice. Proc Natl Acad Sci U S A 94, 8093-8098,
77. Ludviksson, B.R. et al. (1998) Active Wegener's granulomatosis is associated with HLA-DR+CD4+ T cells exhibiting an unbalanced Th1-type T cell cytokine pattern: reversal with IL-10. J Immunol 160, 3602-3609,
78. Csernok, E. et al. (1999) Cytokine profiles in Wegener's granulomatosis: predominance of type 1 (Th1) in the granulomatous inflammation. Arthritis Rheum 42, 742-750,
79. Winek, J. et al. (2004) Frequency of proteinase 3 (PR3)-specific autoreactive T cells determined by cytokine flow cytometry in Wegener's granulomatosis. J Autoimmun 22, 79-85,
80. Brouwer, E. et al. (1994) T cell reactivity to proteinase 3 and myeloperoxidase in patients with Wegener's granulomatosis (WG). Clin Exp Immunol 98, 448-453,
81. *0401 positive patients with necrotizing vasculitis. Blood 92, 3737-3744,
82. Schnabel, A. et al. (1999) T cell receptor vbeta repertoire in bronchoalveolar lavage in Wegener's granulomatosis and sarcoidosis. Int Arch Allergy Immunol 119, 223-230,
83. Offner, H. et al. (1993) Where, when, and how to detect biased expression of disease-relevant V beta genes in rats with experimental autoimmune encephalomyelitis. J Immunol 151, 506-517,
84. Offner, H. et al. (2005) Etanercept plus standard therapy for Wegener's granulomatosis. N Engl J Med 352, 351-361,
85. Offner, H. et al. (2002) Design of the Wegener's Granulomatosis Etanercept Trial (WGET). Control Clin Trials 23, 450-468,
86. Sandborn, W.J. et al. (2001) Etanercept for active Crohn's disease: a randomized, double-blind, placebo-controlled trial. Gastroenterology 121, 1088-1094,
87. Present, D.H. et al. (1999) Infliximab for the treatment of fistulas in patients with Crohn's disease. N Engl J Med 340, 1398-1405,
88. Lugering, A. et al. (2001) Infliximab induces apoptosis in monocytes from patients with chronic active Crohn's disease by using a caspase-dependent pathway. Gastroenterology 121, 1145-1157,
89. Scallon, B.J. et al. (1995) Chimeric anti-TNF-alpha monoclonal antibody cA2 binds recombinant transmembrane TNF-alpha and activates immune effector functions. Cytokine 7, 251-259,
90. Rastetter, W., Molina, A. and White, C.A. (2004) Rituximab: expanding role in therapy for lymphomas and autoimmune diseases. Annu Rev Med 55, 477-503,
91. Uchida, J. et al. (2004) The innate mononuclear phagocyte network depletes B lymphocytes through Fc receptor-dependent mechanisms during anti-CD20 antibody immunotherapy. J Exp Med 199, 1659-1669,
92. Keogh, K.A. et al. (2005) Induction of remission by B lymphocyte depletion in eleven patients with refractory antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum 52, 262-268,
93. Sneller, M.C. (2005) Rituximab and Wegener's granulomatosis: are B cells a target in vasculitis treatment? Arthritis Rheum 52, 1-5,
94. Sneller, M.C. and Fauci, A.S. (1997) Pathogenesis of vasculitis syndromes. Med Clin North Am 81, 221-242,