Skip to main content

Abstract

Microscopic polyangiitis (MPA) belongs to the antineutrophil cytoplasmic autoantibody (ANCA)-associated vasculitides (AAVs). MPA is clinically characterized by small-vessel vasculitis primarily affecting the kidneys and the lungs, but other organs may be involved as well. Renal involvement, which can be the only manifestation, is clinically apparent as rapidly progressive glomerulonephritis and histopathologically as pauci-immune necrotizing and crescentic glomerulonephritis. ANCAs in MPA are mainly directed to myeloperoxidase (MPO-ANCA). Induction treatment consists of cyclophosphamide and steroids followed by azathioprine as maintenance therapy. Other, less toxic therapeutic regimens are being tested.

This is a preview of subscription content, log in via an institution to check access.

Access this chapter

Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 219.00
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 279.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 379.99
Price excludes VAT (USA)
  • Durable hardcover edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info

Tax calculation will be finalised at checkout

Purchases are for personal use only

Institutional subscriptions

Preview

Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.

References

  1. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides. Proposal of an international consensus conference. Arthritis Rheum 1994; 37: 187–92.

    Article  CAS  PubMed  Google Scholar 

  2. Watts RA, Scott DG. Epidemiology of the vasculitides. Semin Respir Crit Care Med 2004; 25: 455–64.

    Article  PubMed  Google Scholar 

  3. Savage C, Winearts C, Evans D, Rees A, Lockwood C. Microscopic polyarteritis: Presentation, pathology and prognosis. Q J Med 1985; 56: 467–83.

    CAS  PubMed  Google Scholar 

  4. Kallenberg CGM, Heeringa P, Stegeman CA. Mechanisms of disease: pathogenesis and treatment of ANCA-associated vasculitides. Nat Clin Pract Rheumatol 2006; 2: 661–70.

    Article  CAS  PubMed  Google Scholar 

  5. Hogan SL, Satterly KK, Dooley MA, et al. Silica exposure in antineutrophil cytoplasmic autoantibody-associated glomerulonephritis and lupus nephritis. J AM Soc Nephrol 2001; 12: 134–42.

    CAS  PubMed  Google Scholar 

  6. Kallenberg CGM, Stegeman CA, Bootsma H, Bijl M, Limburg PC. Quantitation of autoantibodies in systemic autoimmune diseases: Clinically useful? Lupus 2006; 15: 397–402.

    Article  CAS  PubMed  Google Scholar 

  7. Harper L, Savage CO. Leukocyte-endothelial interactions in antineutrophil cytoplasmic antibody-associated systemic vasculitis. Rheum Dis Clin North Am 2001; 27: 887–903.

    Article  CAS  PubMed  Google Scholar 

  8. Xiao H, Heeringa P, Hu P, et al. Antineutrophil cytoplasmic autoantibodies specific for myeloperoxidase cause glomerulonephritis and vasculitis in mice. J Clin Invest 2002; 110: 955–63.

    CAS  PubMed  Google Scholar 

  9. Huugen D, Xiao H, van Esch A, et al. Aggravation of anti-myeloperoxidase antibody-induced glomerulonephritis by bacterial lipopolysaccharide: Role of tumor necrosis factor-alpha. Am J Pathol 2005; 167: 47–58.

    CAS  PubMed  Google Scholar 

  10. Guillevin L, Durand-Gasselin B, Cevallos R, et al. Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 1999; 42: 421–30.

    Article  CAS  PubMed  Google Scholar 

  11. Niles JL, Bottinger EP, Saurina GR, et al. The syndrome of lung hemorrhage and nephritis is usually an ANCA-associated condition. Arch Intern Med 1996; 156: 440–5.

    Article  CAS  PubMed  Google Scholar 

  12. Franssen CFM, Stegeman CA, Kallenberg CGM, Gans ROB, de Jong PE, Hoorntje SJ, Cohen Tervaert JW. Antiproteinase 3- and antimyeloperoxidase-associated vasculitis. Kidney Int 2000; 57: 2195–206.

    Article  CAS  PubMed  Google Scholar 

  13. Bosch X, Guilabert A, Font J. Antineutrophil cytoplasmic antibodies. Lancet 2006; 368: 404–18.

    Article  CAS  PubMed  Google Scholar 

  14. Watts R, Lane S, Hanslik T, et al. Development and validation of a consensus methodology for the classification of the ANCA-associated vasculitides and polyarteritis nodosa for epidemiological studies. Ann Rheum Dis 2007; 66: 222–7.

    Article  PubMed  Google Scholar 

  15. Booth AD, Almond MK, Burns A, et al. Pan-Thames Renal Research Group. Outcome of ANCA-associated renal vasculitis: A 5-year retrospective study. Am J Kidney Dis 2003; 41: 776–84.

    Article  PubMed  Google Scholar 

  16. Bourgarit A, Le Toumelin PH, Pagnoux C, et al. Deaths occurring during the first year after treatment onset for polyarteritis nodosa, microscopic polyangiitis, and Churg-Strauss Syndrome. Medicine 2005; 84: 323–30.

    Article  PubMed  Google Scholar 

  17. Hogan SL, Nachman PH, Falk RJ, et al. Prognostic markers in patients with antineutrophil cytoplasmic autoantibody-associated microscopic polyangiitis and glomerulonephritis. J Am Soc Nephrol 1996; 7: 23–32.

    CAS  PubMed  Google Scholar 

  18. Jayne DR, Gaskin G, Rasmussen N, et al. European Vasculitis Study Group. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 2007; 18: 2180–8.

    Article  CAS  PubMed  Google Scholar 

  19. Jayne D, Rasmussen N, Andrassy K, et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 2003; 349: 36–44.

    Article  CAS  PubMed  Google Scholar 

  20. Jayne D. Part 3: Newer therapies for ANCA-associated vasculitis. Clin Exp Rheumatol 2007; 25(Suppl. 44): S77–9.

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Editor information

Editors and Affiliations

Rights and permissions

Reprints and permissions

Copyright information

© 2008 Humana Press, a part of Springer Science+Business Media, LLC

About this chapter

Cite this chapter

Kallenberg, C.G. (2008). Microscopic Polyangiitis. In: Shoenfeld, Y., Cervera, R., Gershwin, M.E. (eds) Diagnostic Criteria in Autoimmune Diseases. Humana Press. https://doi.org/10.1007/978-1-60327-285-8_17

Download citation

Publish with us

Policies and ethics