Skip to main content
SpringerLink
Log in

Treatment of ANCA-Associated Vasculitides

  • Chapter
  • First Online:
Systemic Vasculitides: Current Status and Perspectives

  • 681 Accesses

Abstract

ANCA-associated vasculitis (AAV) covers a group of systemic necrotizing vasculitides characterized by inflammation of small vessels, some with granuloma, and associated with autoantibodies to neutrophil cytoplasmic proteases (proteinase-3 or myeloperoxidase). Potentially lethal if not promptly diagnosed and treated, AAV in most patients can be induced into remission with the current treatment modalities. However, the risk of relapse remains high, necessitating prolonged immunosuppressive maintenance therapy, whose optimal duration remains undetermined. Herein, we review those treatment modalities for AAV. The findings of most important and recently completed therapeutic studies, including those on rituximab for maintenance, are summarized.

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

Access this chapter

Log in via an institution
Chapter
USD 29.95
Price excludes VAT (USA)
  • Available as PDF
  • Read on any device
  • Instant download
  • Own it forever
eBook
USD 89.00
Price excludes VAT (USA)
  • Available as EPUB and PDF
  • Read on any device
  • Instant download
  • Own it forever
Softcover Book
USD 119.99
Price excludes VAT (USA)
  • Compact, lightweight edition
  • Dispatched in 3 to 5 business days
  • Free shipping worldwide - see info
Hardcover Book
USD 169.99
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

References

  1. Mohammad AJ, Jacobsson LT, Westman KW, Sturfelt G, Segelmark M (2009) Incidence and survival rates in Wegener’s granulomatosis, microscopic polyangiitis, Churg–Strauss syndrome and polyarteritis nodosa. Rheumatology (Oxford) 48(12):1560–1565

    Article  Google Scholar 

  2. Mohammad AJ, Jacobsson LT, Mahr AD, Sturfelt G, Segelmark M (2007) Prevalence of Wegener’s granulomatosis, microscopic polyangiitis, polyarteritis nodosa and Churg–Strauss syndrome within a defined population in southern Sweden. Rheumatology (Oxford) 46(8):1329–1337

    Article  CAS  Google Scholar 

  3. Watts RA, Jolliffe VA, Carruthers DM, Lockwood M, Scott DG (1996) Effect of classification on the incidence of polyarteritis nodosa and microscopic polyangiitis. Arthritis Rheum 39(7):1208–1212

    Article  CAS  PubMed  Google Scholar 

  4. Mahr A, Guillevin L, Poissonnet M, Aymé S (2004) Prevalences of polyarteritis nodosa, microscopic polyangiitis, Wegener’s granulomatosis, and Churg–Strauss syndrome in a French urban multiethnic population in 2000: a capture–recapture estimate. Arthritis Rheum 51(1):92–99

    Article  PubMed  Google Scholar 

  5. Guillevin L, Durand-Gasselin B, Cevallos R, Gayraud M, Lhote F, Callard P et al (1999) Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 42(3):421–430

    Article  CAS  PubMed  Google Scholar 

  6. Comarmond C, Pagnoux C, Khellaf M, Cordier JF, Hamidou M, Viallard JF et al (2013) Eosinophilic granulomatosis with polyangiitis (Churg–Strauss): clinical characteristics and long-term followup of the 383 patients enrolled in the French Vasculitis Study Group cohort. Arthritis Rheum 65(1):270–281

    Article  PubMed  Google Scholar 

  7. Holle JU, Gross WL, Latza U, Nolle B, Ambrosch P, Heller M et al (2011) Improved outcome in 445 patients with Wegener’s granulomatosis in a German vasculitis center over four decades. Arthritis Rheum 63(1):257–266

    Article  PubMed  Google Scholar 

  8. Flossmann O, Berden A, de Groot K, Hagen C, Harper L, Heijl C et al (2011) Long-term patient survival in ANCA-associated vasculitis. Ann Rheum Dis 70(3):488–494

    Article  PubMed  Google Scholar 

  9. Guillevin L, Pagnoux C, Seror R, Mahr A, Mouthon L, Le Toumelin P (2011) The Five-Factor Score revisited: assessment of prognoses of systemic necrotizing vasculitides based on the French Vasculitis Study Group (FVSG) cohort. Medicine (Baltimore) 90(1):19–27

    Article  Google Scholar 

  10. McGregor JG, Hogan SL, Hu Y, Jennette CE, Falk RJ, Nachman PH (2012) Glucocorticoids and relapse and infection rates in anti-neutrophil cytoplasmic antibody disease. Clin J Am Soc Nephrol 7(2):240–247

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  11. Walsh M, Merkel PA, Mahr A, Jayne D (2010) Effects of duration of glucocorticoid therapy on relapse rate in antineutrophil cytoplasmic antibody-associated vasculitis: a meta-analysis. Arthritis Care Res (Hoboken) 62(8):1166–1173

    Article  Google Scholar 

  12. Stone JH, Merkel PA, Spiera R, Seo P, Langford CA, Hoffman GS et al (2010) Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 363(3):221–232

    Article  CAS  PubMed  PubMed Central  Google Scholar 

  13. Pagnoux C, Mahr A, Hamidou MA, Boffa JJ, Ruivard M, Ducroix JP et al (2008) Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N Engl J Med 359(26):2790–2803

    Article  CAS  PubMed  Google Scholar 

  14. Jayne D, Rasmussen N, Andrassy K, Bacon P, Tervaert JW, Dadoniene J et al (2003) A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 349(1):36–44

    Article  CAS  PubMed  Google Scholar 

  15. Guillevin L, Cordier JF, Lhote F, Cohen P, Jarrousse B, Royer I et al (1997) A prospective, multicenter, randomized trial comparing steroids and pulse cyclophosphamide versus steroids and oral cyclophosphamide in the treatment of generalized Wegener’s granulomatosis. Arthritis Rheum 40(12):2187–2198

    Article  CAS  PubMed  Google Scholar 

  16. Haubitz M, Schellong S, Gobel U, Schurek HJ, Schaumann D, Koch KM et al (1998) Intravenous pulse administration of cyclophosphamide versus daily oral treatment in patients with antineutrophil cytoplasmic antibody-associated vasculitis and renal involvement: a prospective, randomized study. Arthritis Rheum 41(10):1835–1844

    Article  CAS  PubMed  Google Scholar 

  17. de Groot K, Harper L, Jayne DR, Flores Suarez LF, Gregorini G, Gross WL et al (2009) Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann Intern Med 150(10):670–680

    Article  PubMed  Google Scholar 

  18. Harper L, Morgan MD, Walsh M, Hoglund P, Westman K, Flossmann O et al (2012) Pulse versus daily oral cyclophosphamide for induction of remission in ANCA-associated vasculitis: long-term follow-up. Ann Rheum Dis 71(6):955–960

    Article  CAS  PubMed  Google Scholar 

  19. Faurschou M, Sorensen IJ, Mellemkjaer L, Loft AG, Thomsen BS, Tvede N et al (2008) Malignancies in Wegener’s granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients. J Rheumatol 35(1):100–105

    CAS  PubMed  Google Scholar 

  20. Le Guenno G, Mahr A, Pagnoux C, Dhote R, Guillevin L (2011) Incidence and predictors of urotoxic adverse events in cyclophosphamide-treated patients with systemic necrotizing vasculitides. Arthritis Rheum 63(5):1435–1445

    Article  PubMed  Google Scholar 

  21. Jones RB, Tervaert JW, Hauser T, Luqmani R, Morgan MD, Peh CA et al (2010) Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N Engl J Med 363(3):211–220

    Article  CAS  PubMed  Google Scholar 

  22. Specks U, Merkel PA, Seo P, Spiera R, Langford CA, Hoffman GS et al (2013) Efficacy of remission-induction regimens for ANCA-associated vasculitis. N Engl J Med 369(5):417–427

    Article  CAS  PubMed  Google Scholar 

  23. Charles P, Bienvenu B, Bonnotte B, Gobert P, Godmer P, Hachulla E et al (2013) Rituximab: recommendations of the French Vasculitis Study Group (FVSG) for induction and maintenance treatments of adult, antineutrophil cytoplasm antibody-associated necrotizing vasculitides. Presse Med 42(10):1317–1330

    Article  PubMed  Google Scholar 

  24. Sanders JS, Slot MC, Stegeman CA (2003) Maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N Engl J Med 349(21):2072–2073; author reply −3

    Google Scholar 

  25. Hiemstra TF, Walsh M, Mahr A, Savage CO, de Groot K, Harper L et al (2010) Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. JAMA 304(21):2381–2388

    Article  CAS  PubMed  Google Scholar 

  26. Guillevin L, Pagnoux C, Karras A, Khouatra C, Aumaitre O, Cohen P et al (2014) Rituximab versus azathioprine for maintenance in ANCA-associated vasculitis. N Engl J Med 371(19):1771–1780

    Article  PubMed  Google Scholar 

  27. Stegeman CA, Tervaert JW, de Jong PE, Kallenberg CG (1996) Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener’s granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med 335(1):16–20

    Article  CAS  PubMed  Google Scholar 

  28. Martinez V, Cohen P, Pagnoux C, Vinzio S, Mahr A, Mouthon L et al (2008) Intravenous immunoglobulins for relapses of systemic vasculitides associated with antineutrophil cytoplasmic autoantibodies: results of a multicenter, prospective, open-label study of twenty-two patients. Arthritis Rheum 58(1):308–317

    Article  CAS  PubMed  Google Scholar 

  29. Jayne DR, Gaskin G, Rasmussen N, Abramowicz D, Ferrario F, Guillevin L et al (2007) Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol 18(7):2180–2188

    Article  CAS  PubMed  Google Scholar 

Download references

Financial Disclosures

Dr. Guillevin reports receiving fees for serving on GlaxoSmithKline and Actelion advisory boards, and lecture fees from Roche, Actelion, Pfizer, CSL Behring, LFB Pharma, and Octapharma. As of January 2014, Dr Guillevin has no conflicts of interest with pharmaceutical companies to disclose.

Author information

Authors and Affiliations

  1. Department of Internal Medicine, Referral Center for Rare Autoimmune and Systemic Diseases, Hôpital Cochin, APHP, Université Paris Descartes, 27, rue du faubourg Saint-Jacques, 75679, Paris, France

    Loïc Guillevin

Authors
  1. Loïc Guillevin
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to Loïc Guillevin .

Editor information

Editors and Affiliations

  1. University of Bari Medical School, Bari, Italy

    Franco Dammacco

  2. University of Bari Medical School, Bari, Italy

    Domenico Ribatti

  3. University of Bari Medical School, Bari, Italy

    Angelo Vacca

Rights and permissions

Reprints and permissions

Copyright information

© 2016 Springer International Publishing Switzerland

About this chapter

Cite this chapter

Guillevin, L. (2016). Treatment of ANCA-Associated Vasculitides. In: Dammacco, F., Ribatti, D., Vacca, A. (eds) Systemic Vasculitides: Current Status and Perspectives. Springer, Cham. https://doi.org/10.1007/978-3-319-40136-2_35

Download citation

Publish with us

Policies and ethics

Search

Navigation