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The Granulomatosis of Wegener’s

Wolfgang L. Gross, MD, PhD, Antje Mueller, PhD, and Julia Holle, MD  |  Issue: May 2011  |  

The authors are part of the Vasculitis Center, Department of Rheumatology and Clinical Immunology, University Hospital Schleswig-Holstein, Campus Lübeck and Klinikum Bad Bramstedt, Germany.

References

  1. Leavitt RY, Fauci AS, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum. 1990;33:1101-1107.
  2. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum. 1994;37:187-192.
  3. Bacon PA. The spectrum of Wegener’s granulomatosis and disease relapse. N Engl J Med. 2005;352:330-332.
  4. Reinhold-Keller E, Beuge N, Latza U, et al. An interdisciplinary approach to the care of patients with Wegener’s granulomatosis: Long-term outcome in 155 patients. Arthritis Rheum. 2000;43:1021-1032.
  5. Xiao H, Heeringa P, Hu P, et al. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidas cause glomerulonephritis and vasculitis in mice. J Clin Invest. 2002;110:955-963.
  6. Stone JH; Wegener’s granulomatosis etanercept trial research group. Limited versus severe Wegener’s granulomatosis: Baseline data on patients in the Wegener’s granulomatosis etanercept trial. Arthritis Rheum. 2003;48:2299-2309.
  7. Rasmussen N, Jayne DR, Abramowicz D, et al. European therapeutic trials in ANCA-associated systemic vasculitis: Disease scoring, consensus regimens and proposed clinical trials. Clin Exp Immunol. 1995;101:S1:29-43.
  8. Hellmich B, Flossman O, Gross WL, et al. EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: Focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis. 2007;66:605-617.
  9. Holle JU, Gross WL, Holl-Ulrich K, et al. Prospective long-term follow-up of patients with localised Wegener’s granulomatosis: Does it occur as persistent disease stage? Ann Rheum Dis. 2010;69:1934-1939.
  10. Pagnoux C, Stubbe M, Lifermann F, et al. Wegener’s granulomatosis strictly and persistently localized to one organ is rare: Assessment of 16 patients from the French vasculitis study group database. J Rheumatol. 2011;38:475-478.
  11. Seo P, Min YI, Holbrook JT, et al. Damage caused by Wegener’s granulomatosis and its treatment: Prospective data from the Wegener’s granulomatosis Etanercept Trial (WGET). Arthritis Rheum. 2005;52:2168-2178.
  12. Little MA, Nightingale P, Verburgh CA, et al. Early mortality in systemic vasculitis: Relative contribution of adverse events and active vasculitis. Ann Rheum Dis. 2010;69:1036-1043.
  13. Muhle C, Reinhold-Keller E, Richter C, et al. MRI of the nasal cavity, the paranasal sinuses and orbits in Wegener’s granulomatosis. Eur Radiol. 1997;7:566-570.
  14. Fujimoto S, Uezono S, Hisanga S, et al. Incidence of ANCA-associated primary renal vasculitis in the Miyazaki Prefecture: The first population-based, retrospective, epidemiologic survey in Japan. Clin Am Soc Nephrol. 2006;1:1016-1022.
  15. Jagiello P, Aries P, Arning L, et al. The PTPN22 620W allele is a risk factor for Wegener’s granulomatosis. Arthritis Rheum. 2005;12:4039-4043.
  16. Carr EJ, Niederer HA, Williams J, et al. Confirmation of the genetic association of CTLA4 and PTPN22 with ANCA-associated vasculitis. BMC Med Genet. 2009;10:121.
  17. Heckmann M, Holle JU, Arning L, et al. The Wegener’s granulomatosis quantative trait locus on chromosome 6p21.3 as characterized by tagSNP genotyping. Ann Rheum Dis. 2008;67:972-979.
  18. Arning L, Holle JU, Harper L, et al. Are there specific genetic risk factors for the different forms of ANCA-associated vasculitis? Ann Rheum Dis. 2011;70:707-708.
  19. Lie JT. Wegener’s granulomatosis: Histological documentation of common and uncommon manifestations in 216 patients. Vasa. 1997;26:261-270.
  20. Holl-Ulrich K. Histopathology of systemic vasculitis. Pathologe. 2010;31:67-76.
  21. Mueller A, Holl-Ulrich K, Lamprecht P, et al. Germinal centre-like structures in Wegener’s granuloma: The morphological basis for autoimmunity? Rheumatology. 2008;47:1111-1113.
  22. Remijsen Q, Berghe TV, Wirawan E, et al. Neutrophil extracellular trap cell death requires both autophagy and superoxide generation. Cell Res. 2011;21:290-304.
  23. Papayannopoulos V, Zychlinsky A. NETs: A new strategy for using old weapons. Trends Immunol. 2009;30:513-521.
  24. Kessenbrock K, Krumbholz M, Schönermarck U, et al. Netting neutrophils in autoimmune small-vessel vasculitis. Nat Med. 2009;15:623-625.
  25. Urban C, Ermert D, Schmid M, et al. Neutrophil extracellular traps contain calprotectin, a cytosolic protein complex involved in host defense against Candida albicans. PLoS Pathog. 2009;5:e1000639.
  26. Scapini P, Bazzoni F, Cassatella M. Regulation of B-cell-activating factor (BAFF)/B lymphocyte stimulator (BLys) expression in human neutrophils. Immun Lett. 2008;116:1-6.
  27. Mueller A, Voswinkel J, Hallof A, et al. Immunophenotypic characterization of germinal centre-like structures in Wegener’s granulomatosis. APMIS. 117:126 [abstract].
  28. Aries P, Lamprecht P, Gross WL. Wegener’s granulomatosis: A view from the granulomatous side of the disease. IMAJ. 2005;7:768-773.
  29. Voswinkel J, Mueller A, Kraemer JA, et al. B lymphocyte maturation in Wegener’s granulomatosus: A comparative analysis of VH genes from endonasal lesions. Ann Rheum Dis. 2006;65:859-864.
  30. Voswinkel J, Assmann G, Held G, et al. Single cell analysis of B lymphocytes from Wegener’s granulomatosis: B cell receptors display affinity maturation within the granulomatous lesions. Clin Exp Immunol. 2008;154:339-345.
  31. Thurner L, Müller A, Cerutti M, et al. Wegener’s granuloma harbors B lymphocytes with specificities against a proinflammatory membrane protein and a tetraspanin. J Autoimmun. 2011;36:87-90.
  32. Müller A, Trabandt A, Gloeckner-Hofmann K, et al. Localized Wegener’s granulomatosis: Predominance of CD26 and IFN-gamma expression. J Pathol. 2000;192:113-120.
  33. Csernok E, Ai M, Gross WL, et al. Wegener autoantigen induces maturation of dendritic cells and licences them for Th1 priming via the protease-activated receptor-2 pathway. Blood. 2006;107:4440-4448.
  34. Balding CEJ, Howie AJ, Drake-Lee AB, et al. Th2 dominance in nasal mucosa in patients with Wegener’s granulomatosis. Clin Exp Immunol. 2001;125:332-339.
  35. Abdulahad WH, Lamprecht P, Kallenberg CGM. T-helper-cells as new players in ANCA-associated vasculitides. Arthritis Res Ther. Submitted.
  36. Kesel N, Laudien M, Holl-Ulrich K, et al. Xenografted nasal mucosa from Wegener’s granulomatosis patients induces destruction of implanted human cartilage in immunodeficient mice. Arthritis Rheum. 2010;62(10 Suppl):S283 [abstract].
  37. Aries PM, Both M. Images in clinical medicine. Destructive eye lesions in Wegener’s granulomatosis. New Engl J Med. 2005; 352:392.
  38. Holl-Ulrich K, Both M, Gottschlich S, Gross WL, Aries PM, Lamprecht P. Clinical images: Saddle nose deformity caused by destructive granulomatous inflammation in Wegener’s granulomatosis. Arthritis Rheum. 2008;58:834.

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