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BioMed Research International
Volume 2015 (2015), Article ID 402826, 8 pages
http://dx.doi.org/10.1155/2015/402826
Review Article

Pauci-Immune Crescentic Glomerulonephritis: An ANCA-Associated Vasculitis

Division of Nephrology, Staten Island University Hospital, 475 Seaview Avenue, Staten Island, NY 10305, USA

Received 3 September 2015; Revised 15 November 2015; Accepted 17 November 2015

Academic Editor: Monica Carmosino

Copyright © 2015 Rafeel Syed et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Linked References

  1. R. J. Glassock, S. G. Adler, H. J. Ward et al., “Primary glomerular diseases,” in The Kidney, B. M. Brenner and F. C. Rector, Eds., pp. 1182–1279, Saunders, Philadelphia, Pa, USA, 4th edition, 1991. View at Google Scholar
  2. J. Charles Jennette, R. J. Falk, and J. G. McGregor, “Renal and systemic valculitis,” in Comprehensive Clinical Nephrology, Elsevier, 5th edition, 2015. View at Google Scholar
  3. S. Watnick and T. Dirkx, “Kidney disease,” in Current Medical Diagnosis & Treatment 2015, M. A. Papadakis, S. J. McPhee, and M. W. Rabow, Eds., McGraw-Hill, New York, NY, USA, 2014. View at Google Scholar
  4. J. C. Jennette, J. L. Olson, M. M. Schwartz, and F. G. Silva, “Pauci-immune and ANCA-mediated crescentic glomerulonephritis and vasculitis,” in Heptinstall's Pathology of the Kidney, vol. 1, chapter 14, 6th edition, 2007. View at Google Scholar
  5. P. Seo and J. H. Stone, “The antineutrophil cytoplasmic antibody-associated vasculitides,” The American Journal of Medicine, vol. 117, no. 1, pp. 39–50, 2004. View at Publisher · View at Google Scholar · View at Scopus
  6. U. Eisenberger, F. Fakhouri, P. Vanhille et al., “ANCA-negative pauci-immune renal vasculitis: histology and outcome,” Nephrology Dialysis Transplantation, vol. 20, no. 7, pp. 1392–1399, 2005. View at Publisher · View at Google Scholar · View at Scopus
  7. M. Haas and J. A. Eustace, “Immune complex deposits in ANCA-associated crescentic glomerulonephritis: a study of 126 cases,” Kidney International, vol. 65, no. 6, pp. 2145–2152, 2004. View at Publisher · View at Google Scholar · View at Scopus
  8. J. B. Levy, T. Hammad, A. Coulthart, T. Dougan, and C. D. Pusey, “Clinical features and outcome of patients with both ANCA and anti-GBM antibodies,” Kidney International, vol. 66, no. 4, pp. 1535–1540, 2004. View at Publisher · View at Google Scholar · View at Scopus
  9. N. Kambham, “Crescentic glomerulonephritis: an update on pauci-immune and anti-GBM diseases,” Advances in Anatomic Pathology, vol. 19, no. 2, pp. 111–124, 2012. View at Publisher · View at Google Scholar · View at Scopus
  10. F. Ferrario, A. Vanzati, and F. Pagni, “Pathology of ANCA-associated vasculitis,” Clinical and Experimental Nephrology, vol. 17, no. 5, pp. 652–658, 2013. View at Publisher · View at Google Scholar · View at Scopus
  11. A. E. Berden, F. Ferrario, E. C. Hagen et al., “Histopathologic classification of ANCA-associated glomerulonephritis,” Journal of the American Society of Nephrology, vol. 21, no. 10, pp. 1628–1636, 2010. View at Publisher · View at Google Scholar · View at Scopus
  12. S. L. Ford, K. R. Polkinghorne, A. Longano et al., “Histopathologic and clinical predictors of kidney outcomes in ANCA-associated vasculitis,” American Journal of Kidney Diseases, vol. 63, no. 2, pp. 227–235, 2014. View at Publisher · View at Google Scholar · View at Scopus
  13. T. Iwakiri, S. Fujimoto, K. Kitagawa et al., “Validation of a newly proposed histopathological classification in Japanese patients with anti-neutrophil cytoplasmic antibody-associated glomerulonephritis,” BMC Nephrology, vol. 14, article 125, 2013. View at Publisher · View at Google Scholar · View at Scopus
  14. E. Nohr, L. Girard, M. James, and H. Benediktsson, “Validation of a histopathologic classification scheme for antineutrophil cytoplasmic antibody-associated glomerulonephritis,” Human Pathology, vol. 45, no. 7, pp. 1423–1429, 2014. View at Publisher · View at Google Scholar · View at Scopus
  15. D.-Y. Chang, L.-H. Wu, G. Liu, M. Chen, C. G. M. Kallenberg, and M.-H. Zhao, “Re-evaluation of the histopathologic classification of ANCA-associated glomerulonephritis: a study of 121 patients in a single center,” Nephrology Dialysis Transplantation, vol. 27, no. 6, pp. 2343–2349, 2012. View at Publisher · View at Google Scholar · View at Scopus
  16. M. El-Ters, U. Muthyala, M. D. Philipneri, F. A. Hussein, and K. L. Lentine, “Immune-complex deposits in ‘pauci-immune’ glomerulonephritis: a case report and brief review of recent literature,” Archives of Medical Science, vol. 6, no. 4, pp. 633–637, 2010. View at Publisher · View at Google Scholar · View at Scopus
  17. I. Neumann, H. Regele, R. Kain, R. Birck, and F. T. Meisl, “Glomerular immune deposits are associated with increased proteinuria in patients with ANCA-associated crescentic nephritis,” Nephrology Dialysis Transplantation, vol. 18, no. 3, pp. 524–531, 2003. View at Publisher · View at Google Scholar · View at Scopus
  18. E. Brouwer, M. G. Huitema, P. A. Klok et al., “Antimyeloperoxidase-associated proliferative glomerulonephritis: an animal model,” Journal of Experimental Medicine, vol. 177, no. 4, pp. 905–914, 1993. View at Publisher · View at Google Scholar · View at Scopus
  19. J. J. Yang, J. C. Jennette, and R. J. Falk, “Immune complex glomerulonephritis is induced in rats immunized with heterologous myeloperoxidase,” Clinical and Experimental Immunology, vol. 97, no. 3, pp. 466–473, 1994. View at Google Scholar · View at Scopus
  20. M. Haas, J. Jafri, S. M. Bartosh, S. L. Karp, S. G. Adler, and S. M. Meehan, “ANCA-associated crescentic glomerulonephritis with mesangial IgA deposits,” American Journal of Kidney Diseases, vol. 36, no. 4, pp. 709–718, 2000. View at Publisher · View at Google Scholar · View at Scopus
  21. M. Haas, “Incidental healed postinfectious glomerulonephritis: a study of 1012 renal biopsy specimens examined by electron microscopy,” Human Pathology, vol. 34, no. 1, pp. 3–10, 2003. View at Publisher · View at Google Scholar
  22. R. Jimbo, Y. Ubara, T. Tagami et al., “A pediatric occurrence of crescentic glomerulonephritis associated with antineutrophil cytoplasmic antibodies and mesangial IgA deposits,” Clinical Nephrology, vol. 68, no. 2, pp. 104–108, 2007. View at Publisher · View at Google Scholar · View at Scopus
  23. J. C. Jennette, “Rapidly progressive crescentic glomerulonephritis,” Kidney International, vol. 63, no. 3, pp. 1164–1177, 2003. View at Publisher · View at Google Scholar · View at Scopus
  24. P. J. Bansal and M. C. Tobin, “Neonatal microscopic polyangiitis secondary to transfer of maternal myeloperoxidase-antineutrophil cytoplasmic antibody resulting in neonatal pulmonary hemorrhage and renal involvement,” Annals of Allergy, Asthma and Immunology, vol. 93, no. 4, pp. 398–401, 2004. View at Publisher · View at Google Scholar · View at Scopus
  25. H. Xiao, P. Heeringa, P. Hu et al., “Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice,” Journal of Clinical Investigation, vol. 110, no. 7, pp. 955–963, 2002. View at Publisher · View at Google Scholar · View at Scopus
  26. C. G. M. Kallenberg, “Pathogenesis of ANCA-associated vasculitis, an update,” Clinical Reviews in Allergy and Immunology, vol. 41, no. 2, pp. 224–231, 2011. View at Publisher · View at Google Scholar · View at Scopus
  27. H. Pfister, M. Ollert, L. F. Fröhlich et al., “Antineutrophil cytoplasmic autoantibodies against the murine homolog of proteinase 3 (Wegener autoantigen) are pathogenic in vivo,” Blood, vol. 104, no. 5, pp. 1411–1418, 2004. View at Publisher · View at Google Scholar · View at Scopus
  28. R. J. Falk, R. S. Terrell, L. A. Charles, and J. C. Jennette, “Anti-neutrophil cytoplasmic autoantibodies induce neutrophils to degranulate and produce oxygen radicals in vitro,” Proceedings of the National Academy of Sciences of the United States of America, vol. 87, no. 11, pp. 4115–4119, 1990. View at Publisher · View at Google Scholar · View at Scopus
  29. L. A. Charles, M. L. R. Caldas, R. J. Falk, R. S. Terrell, and J. C. Jennette, “Antibodies against granule proteins activate neutrophils in vitro,” Journal of Leukocyte Biology, vol. 50, no. 6, pp. 539–546, 1991. View at Google Scholar · View at Scopus
  30. A. J. Porges, P. B. Redecha, W. T. Kimberly, E. Csernok, W. L. Gross, and R. P. Kimberly, “Anti-neutrophil cytoplasmic antibodies engage and activate human neutrophils via Fc gamma RIIa,” The Journal of Immunology, vol. 153, no. 3, pp. 1271–1280, 1994. View at Google Scholar
  31. A. H. Mulder, P. Heeringa, E. Brouwer, P. C. Limburg, and C. G. Kallenberg, “Activation of granulocytes by anti-neutrophil cytoplasmic antibodies (ANCA): a Fc gamma RII-dependent process,” Clinical & Experimental Immunology, vol. 98, no. 2, pp. 270–278, 1994. View at Google Scholar
  32. R. Kettritz, J. C. Jennette, and R. J. Falk, “Crosslinking of ANCA-antigens stimulates superoxide release by human neutrophils,” Journal of the American Society of Nephrology, vol. 8, no. 3, pp. 386–394, 1997. View at Google Scholar · View at Scopus
  33. B. H. Ewert, J. C. Jennette, and R. J. Falk, “Anti-myeloperoxidase antibodies stimulate neutrophils to damage human endothelial cells,” Kidney International, vol. 41, no. 2, pp. 375–383, 1992. View at Publisher · View at Google Scholar · View at Scopus
  34. K. Kessenbrock, M. Krumbholz, U. Schönermarck et al., “Netting neutrophils in autoimmune small-vessel vasculitis,” Nature Medicine, vol. 15, no. 6, pp. 623–625, 2009. View at Publisher · View at Google Scholar · View at Scopus
  35. A. K. Gupta, M. B. Joshi, M. Philippova et al., “Activated endothelial cells induce neutrophil extracellular traps and are susceptible to NETosis-mediated cell death,” FEBS Letters, vol. 584, no. 14, pp. 3193–3197, 2010. View at Publisher · View at Google Scholar · View at Scopus
  36. O. O. Rowaiye, M. Kusztal, and M. Klinger, “The kidneys and ANCA-associated vasculitis: from pathogenesis to diagnosis,” Clinical Kidney Journal, vol. 8, no. 3, pp. 343–350, 2015. View at Publisher · View at Google Scholar
  37. H. Xiao, A. Schreiber, P. Heeringa, R. J. Falk, and J. C. Jennette, “Alternative complement pathway in the pathogenesis of disease mediated by anti-neutrophil cytoplasmic autoantibodies,” American Journal of Pathology, vol. 170, no. 1, pp. 52–64, 2007. View at Publisher · View at Google Scholar · View at Scopus
  38. U. Schönermarck, E. Csernok, and W. L. Gross, “Pathogenesis of anti-neutrophil cytoplasmic antibody-associated vasculitis: challenges and solutions 2014,” Nephrology Dialysis Transplantation, vol. 30, supplement 1, pp. i46–i52, 2015. View at Publisher · View at Google Scholar
  39. C. Franssen, R. Gans, C. Kallenberg, C. Hageluken, and S. Hoorntje, “Disease spectrum of patients with antineutrophil cytoplasmic autoantibodies of defined specificity: distinct differences between patients with anti-proteinase 3 and anti-myeloperoxidase autoantibodies,” Journal of Internal Medicine, vol. 244, no. 3, pp. 209–216, 1998. View at Publisher · View at Google Scholar · View at Scopus
  40. R. Kain, M. Exner, R. Brandes et al., “Molecular mimicry in pauci-immune focal necrotizing glomerulonephritis,” Nature Medicine, vol. 14, no. 10, pp. 1088–1096, 2008. View at Publisher · View at Google Scholar · View at Scopus
  41. R. Kain, H. Tadema, E. F. McKinney et al., “High prevalence of autoantibodies to hLAMP-2 in anti-neutrophil cytoplasmic antibody-associated vasculitis,” Journal of the American Society of Nephrology, vol. 23, no. 3, pp. 556–566, 2012. View at Publisher · View at Google Scholar · View at Scopus
  42. A. J. Roth, M. C. Brown, R. N. Smith et al., “Anti-LAMP-2 antibodies are not prevalent in patients with antineutrophil cytoplasmic autoantibody glomerulonephritis,” Journal of the American Society of Nephrology, vol. 23, no. 3, pp. 545–555, 2012. View at Publisher · View at Google Scholar · View at Scopus
  43. R. Kain and A. J. Rees, “What is the evidence for antibodies to LAMP-2 in the pathogenesis of ANCA associated small vessel vasculitis?” Current Opinion in Rheumatology, vol. 25, no. 1, pp. 26–34, 2013. View at Publisher · View at Google Scholar · View at Scopus
  44. J. Hao, C. Wang, S.-J. Gou, M.-H. Zhao, and M. Chen, “The association between anti-plasminogen antibodies and disease activity in ANCA-associated vasculitis,” Rheumatology, vol. 53, no. 2, pp. 300–306, 2014. View at Publisher · View at Google Scholar · View at Scopus
  45. A. E. Berden, S. L. Nolan, H. L. Morris et al., “Anti-plasminogen antibodies compromise fibrinolysis and associate with renal histology in ANCA-associated vasculitis,” Journal of the American Society of Nephrology, vol. 21, no. 12, pp. 2169–2179, 2010. View at Publisher · View at Google Scholar · View at Scopus
  46. C. A. Stegeman, J. W. Cohen Tervaert, W. J. Sluiter, W. L. Manson, P. E. De Jong, and C. G. M. Kallenberg, “Association of chronic nasal carriage of Staphylococcus aureus and higher relapse rates in Wegener granulomatosis,” Annals of Internal Medicine, vol. 120, no. 1, pp. 12–17, 1994. View at Publisher · View at Google Scholar · View at Scopus
  47. K. Zycinska, K. A. Wardyn, T. M. Zielonka, R. Krupa, and W. Lukas, “Co-trimoxazole and prevention of relapses of PR3-ANCA positive vasculitis with pulmonary involvement,” European Journal of Medical Research, vol. 14, supplement 4, pp. 265–267, 2009. View at Publisher · View at Google Scholar · View at Scopus
  48. A. D. Salama and C. D. Pusey, “Shining a LAMP on pauci-immune focal segmental glomerulonephritis,” Kidney International, vol. 76, no. 1, pp. 15–17, 2009. View at Publisher · View at Google Scholar · View at Scopus
  49. M. M. Van Timmeren and P. Heeringa, “Pathogenesis of ANCA-associated vasculitis: recent insights from animal models,” Current Opinion in Rheumatology, vol. 24, no. 1, pp. 8–14, 2012. View at Publisher · View at Google Scholar · View at Scopus
  50. A. M. Coughlan, S. J. Freeley, and M. G. Robson, “Animal models of anti-neutrophil cytoplasmic antibody-associated vasculitis,” Clinical and Experimental Immunology, vol. 169, no. 3, pp. 229–237, 2012. View at Publisher · View at Google Scholar · View at Scopus
  51. S. L. Hogan, K. K. Satterly, M. A. Dooley, P. H. Nachman, J. C. Jennette, and R. J. Falk, “Silica exposure in anti-neutrophil cytoplasmic autoantibody-associated glomerulonephritis and lupus nephritis,” Journal of the American Society of Nephrology, vol. 12, no. 1, pp. 134–142, 2001. View at Google Scholar · View at Scopus
  52. Z. Rihova, D. Maixnerova, E. Jancova et al., “Silica and asbestos exposure in ANCA-associated vasculitis with pulmonary involvement,” Renal Failure, vol. 27, no. 5, pp. 605–608, 2005. View at Publisher · View at Google Scholar · View at Scopus
  53. T. Kanda, H. Tanio, C. Wu, H. Nishihara, F. Nogaki, and T. Ono, “Churg-Strauss syndrome with severe granulomatous angiitis and crescentic glomerulonephritis, which developed during therapy with a leukotriene receptor antagonist,” Clinical and Experimental Nephrology, vol. 14, no. 6, pp. 602–607, 2010. View at Publisher · View at Google Scholar · View at Scopus
  54. M. Suneja, S. Baiswar, and S. A. Vogelgesang, “Hydralazine associated pauci-immune glomerulonephritis,” Journal of Clinical Rheumatology, vol. 20, no. 2, pp. 99–102, 2014. View at Publisher · View at Google Scholar · View at Scopus
  55. S. Morita, Y. Ueda, and K. Eguchi, “Anti-thyroid drug-induced ANCA-associated vasculitis: a case report and review of the literature,” Endocrine Journal, vol. 47, no. 4, pp. 467–470, 2000. View at Publisher · View at Google Scholar · View at Scopus
  56. H. K. Choi, P. A. Merkel, A. M. Walker, and J. L. Niles, “Drug-associated antineutrophil cytoplasmic antibody-positive vasculitis: prevalence among patients with high titers of antimyeloperoxidase antibodies,” Arthritis and Rheumatism, vol. 43, no. 2, pp. 405–413, 2000. View at Google Scholar · View at Scopus
  57. J. C. Jennette, R. J. Falk, and A. H. Gasim, “Pathogenesis of antineutrophil cytoplasmic autoantibody vasculitis,” Current Opinion in Nephrology and Hypertension, vol. 20, no. 3, pp. 263–270, 2011. View at Publisher · View at Google Scholar · View at Scopus
  58. W. F. Pendergraft III, G. A. Preston, R. R. Shah et al., “Autoimmunity is triggered by cPR-3(105–201), a protein complementary to human autoantigen proteinase-3,” Nature Medicine, vol. 10, no. 1, pp. 72–79, 2004. View at Publisher · View at Google Scholar · View at Scopus
  59. P. Hewins, F. Belmonte, J. C. Jennette, R. J. Falk, and G. A. Preston, “Longitudinal studies of patients with ANCA vasculitis demonstrate concurrent reactivity to complementary PR3 protein segments cPR3m and cPR3C and with no reactivity to cPR3N,” Autoimmunity, vol. 44, no. 2, pp. 98–106, 2011. View at Publisher · View at Google Scholar · View at Scopus
  60. P. A. Lyons, T. F. Rayner, S. Trivedi et al., “Genetically distinct subsets within ANCA-associated vasculitis,” The New England Journal of Medicine, vol. 367, no. 3, pp. 214–223, 2012. View at Google Scholar
  61. G. Xie, D. Roshandel, R. Sherva et al., “Association of granulomatosis with polyangiitis (Wegener's) with HLA-DPB104 and SEMA6A gene variants: evidence from genome-wide analysis,” Arthritis & Rheumatism, vol. 65, no. 9, pp. 2457–2468, 2013. View at Publisher · View at Google Scholar · View at Scopus
  62. V. Witko-Sarsat, P. Lesavre, S. Lopez et al., “A large subset of neutrophils expressing membrane proteinase 3 is a risk factor for vasculitis and rheumatoid arthritis,” Journal of the American Society of Nephrology, vol. 10, no. 6, pp. 1224–1233, 1999. View at Google Scholar · View at Scopus
  63. P. Jagiello, P. Aries, L. Arning et al., “The PTPN22 620W allele is a risk factor for Wegener's granulomatosis,” Arthritis and Rheumatism, vol. 52, no. 12, pp. 4039–4043, 2005. View at Publisher · View at Google Scholar · View at Scopus
  64. D. Martorana, F. Maritati, G. Malerba et al., “PTPN22 R620W polymorphism in the ANCA-associated vasculitides,” Rheumatology, vol. 51, no. 5, Article ID ker446, pp. 805–812, 2012. View at Publisher · View at Google Scholar · View at Scopus
  65. R. Giscombe, X. Wang, D. Huang, and A. K. Lefvert, “Coding sequence 1 and promoter single nucleotide polymorphisms in the CTLA-4 gene in Wegener's granulomatosis,” Journal of Rheumatology, vol. 29, no. 5, pp. 950–953, 2002. View at Google Scholar · View at Scopus
  66. P. Lamprecht, S. Wieczorek, J. T. Epplen, P. Ambrosch, and C. G. M. Kallenberg, “Granuloma formation in ANCA-associated vasculitides,” APMIS, vol. 117, supplement s127, no. 127, pp. 32–36, 2009. View at Publisher · View at Google Scholar · View at Scopus
  67. M. D. Morgan, C. J. Day, K. P. Piper et al., “Patients with Wegener's granulomatosis demonstrate a relative deficiency and functional impairment of T-regulatory cells,” Immunology, vol. 130, no. 1, pp. 64–73, 2010. View at Publisher · View at Google Scholar · View at Scopus
  68. M. E. Free, D. O. Bunch, J. A. McGregor et al., “Patients with antineutrophil cytoplasmic antibody-associated vasculitis have defective Treg cell function exacerbated by the presence of a suppression-resistant effector cell population,” Arthritis and Rheumatism, vol. 65, no. 7, pp. 1922–1933, 2013. View at Publisher · View at Google Scholar · View at Scopus
  69. B. Wilde, M. Thewissen, J. Damoiseaux et al., “Regulatory B cells in ANCA-associated vasculitis,” Annals of the Rheumatic Diseases, vol. 72, no. 8, pp. 1416–1419, 2013. View at Publisher · View at Google Scholar · View at Scopus
  70. N. Kesel, D. Khler, L. Herich et al., “Cartilage destruction in granulomatosis with polyangiitis (Wegener's granulomatosis) is mediated by human fibroblasts after transplantation into immunodeficient mice,” The American Journal of Pathology, vol. 180, no. 5, pp. 2144–2155, 2012. View at Publisher · View at Google Scholar · View at Scopus
  71. C. Rahmattulla, R. A. de Lind van Wijngaarden, A. E. Berden et al., “Renal function and ear, nose, throat involvement in anti-neutrophil cytoplasmic antibody-associated vasculitis: prospective data from the European Vasculitis Society clinical trials,” Rheumatology, vol. 54, no. 5, pp. 899–907, 2015. View at Publisher · View at Google Scholar