Skip to main content
SpringerLink
Log in

ANCA („anti-neutrophil cytoplasm antibody“)-assoziierte Vaskulitiden

ANCA-associated vasculitis

  • CME Zertifizierte Fortbildung
  • Published:
Zeitschrift für Rheumatologie Aims and scope Submit manuscript

Zusammenfassung

Die Granulomatose mit Polyangiitis, die mikroskopische Polyangiitis und die eosinophile Granulomatose mit Polyangiitis werden zu den ANCA („anti-neutrophil cytoplasm antibody“)-assoziierten Vaskulitiden (AAV) zusammengefasst. Ihnen gemeinsam ist die Vaskulitis kleiner bis mittelgroßer Gefäße, die in fast jedem Organ auftreten kann. Die Therapie der AAV erfolgt heute in Abhängigkeit von Stadium und Aktivität initial durch eine remissionsinduzierende und nachfolgend durch eine remissionserhaltende Therapie auf der Basis kontrollierter Studien. Neben Glukokortikoiden stellen konventionelle Immunsuppressiva wie Methotrexat oder Cyclophosphamid Eckpfeiler der Therapie dar, wobei auch Biologika (z. B. Rituximab) bereits als neue Optionen eingesetzt werden.

Abstract

Granulomatosis with polyangiitis, microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis belong to the anti-neutrophil cytoplasmic antibodies (ANCA) associated vasculitides (AAV). They share the feature of vasculitic manifestations in small to medium-size vessel beds which can occur in nearly any organ system. The treatment of AAV is dependent on stage and activity and is carried out on the basis of randomized controlled trials with an initial remission induction regimen followed by maintenance treatment. Apart from glucocorticoids, conventional immunosuppressants are the basis of treatment whereby biologics, such as rituximab have emerged as new treatment options.

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

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Literatur

  1. Fries JF, Hunder GG, Bloch DA et al (1990) The American College of Rheumatology 1990 criteria for the classification of vasculitis. Summary. Arthritis Rheum 33:1135–1136

    Article  PubMed  CAS  Google Scholar 

  2. Jennette JC, Falk RJ, Bacon PA et al (2013) 2012 revised international Chapel Hill consensus conference nomenclature of vasculitides. Arthritis Rheum 65:1–11

    Article  PubMed  CAS  Google Scholar 

  3. Leavitt RY, Fauci AS, Bloch DA et al (1990) The American College of Rheumatology 1990 criteria for the classification of Wegener’s granulomatosis. Arthritis Rheum 33:1101–1107

    Article  PubMed  CAS  Google Scholar 

  4. Masi AT, Hunder GG, Lie JT et al (1990) The American College of Rheumatology 1990 criteria for the classification of Churg Strauss syndrome (allergic granulomatosis and angiitis). Arthritis Rheum 33:1094–1100

    Article  PubMed  CAS  Google Scholar 

  5. Hellmich B, Flossmann O, Gross WL et al (2007) EULAR recommendations for conducting clinical studies and/or clinical trials in systemic vasculitis: focus on anti-neutrophil cytoplasm antibody-associated vasculitis. Ann Rheum Dis 66:605–617

    Article  PubMed  CAS  Google Scholar 

  6. Holle JU, Gross WL, Holl-Ulrich K et al (2010) Prospective long-term follow-up of patients with localised Wegener’s granulomatosis: Does it occur as persistent disease stage? Ann Rheum Dis 69:1934–1939

    Article  PubMed  Google Scholar 

  7. Comarmond C, Pagnoux C, Khellaf M et al (2013) Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome) – clinical characteristics and long-term follow-up of the 383 patients enrolled in the FVSG cohort. Arthritis Rheum 65:270–281

    Article  PubMed  Google Scholar 

  8. Moosig F, Bremer JP, Hellmich B et al (2012) A vasculitis centre based management strategy leads to improved outcome in eosinophilic granulomatosis with polyangiitis (Churg-Strauss, EGPA): monocentric experiences in 150 patients. Ann Rheum Dis [Epub ahead of print]

  9. Sablé-Fourtassou R, Cohen P, Mahr A et al (2005) Antineutrophil cytoplasmic antibodies and the Churg-Strauss syndrome. Ann Intern Med 143:623–638

    Article  Google Scholar 

  10. Sinico RA, Di Toma L, Maggiore U et al (2005) Prevalence and clinical significance of antineutrophil cytoplasmic antibodies in Churg-Strauss syndrome. Arthritis Rheum 52:2926–2935

    Article  PubMed  CAS  Google Scholar 

  11. Guillevin L, Durand-Gasselin B, Cevallos R et al (1999) Microscopic polyangiitis: clinical and laboratory findings in eighty-five patients. Arthritis Rheum 42:421–430

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  Google Scholar 

  13. Reinhold-Keller E, Herlyn K, Wagner-Bastmeyer R et al (2005) Stable incidence of primary systemic vasculitides over five years: results from the German vasculitis registry. Arthritis Rheum 53:93–99

    Article  PubMed  Google Scholar 

  14. Savige J, Gillis D, Benson E et al (1999) International consensus statement on testing and reporting of antineutrophil cytoplasmatic antibodies (ANCA). Am J Clin Pathol 111:507–513

    PubMed  CAS  Google Scholar 

  15. Mukhtyar C, Guillevin L, Cid MC et al (2009) EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis 68:310–317

    Article  PubMed  CAS  Google Scholar 

  16. Stone JH, Merkel PA, Spiera R et al (2010) Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N Engl J Med 363:221–232

    Article  PubMed  CAS  Google Scholar 

  17. Pagnoux C, Mahr A, Hamidou MA et al (2008) Azathioprine or methotrexate for ANCA-associated vasculitis. N Engl J Med 359:2790–2803

    Article  PubMed  CAS  Google Scholar 

  18. Hiemstra TF, Walsh M, Mahr A et al (2010) Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. JAMA 304:2381–2388

    Article  PubMed  CAS  Google Scholar 

  19. Vaglio A, Moosig F, Zwerina J (2012) Churg-Strauss syndrome: update on pathophysiology and treatment. Curr Opin Rheumatol 24:24–30

    Article  PubMed  CAS  Google Scholar 

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

    Article  PubMed  CAS  Google Scholar 

  21. Little MA, Nightingale P, Verburgh CA et al (2012) Early mortality in systemic vasculitis: relative contribution of adverse events and active vasculitis. Ann Rheum Dis 69:1036–1043

    Article  Google Scholar 

  22. Bourgarit A, Le Toumelin P, Pagnoux C et al (2005) Deaths occurring during the first year after treatment onset for polyarteriitis nodosa, microscopic polyangiitis and Churg-Strauss syndrome: a retrospective analysis of causes and factors predictive of mortality based on 595 patients. Medicine 84:323–330

    Article  PubMed  Google Scholar 

  23. Metzler C, Hellmich B, Gause A et al (2004) Churg Strauss syndrome – successful induction of remission with methotrexate and unexpected high cardiac and pulmonary relapse ratio during maintenance treatment. Clin Exp Rheumatol 22(6 Suppl 36):S52–S61

    PubMed  CAS  Google Scholar 

  24. Ribi C, Cohen P, Mahr A et al (2008) Treatment of Churg-Strauss syndrome without poor-prognosis factors: a multicenter, prospective, randomized open-label study of seventy-two patients. Arthritis Rheum 58:586–594

    Article  PubMed  CAS  Google Scholar 

  25. Cohen P, Pagnoux C, Mahr A et al (2007) Churg-Strauss syndrome with poor-prognosis factors: a prospective multicenter trial comparing glucocorticoids and six or twelve cycloposphamide pulses in forty-eight patients. Arthritis Rheum 57:676–693

    Article  Google Scholar 

  26. Moosig F, Gross WL, Herrmann K et al (2011) Targeting interleukin-5 in refractory and relapsing Churg-Strauss syndrome. Ann Intern Med 155:341–343

    Article  PubMed  Google Scholar 

  27. Mahr A, Katsahian S, Varet H et al (2012) Revisiting the classification of clinical phenotypes of anti-neutrophil cytoplasmic antibody associated vasculitis: a meta-analysis. Ann Rheum Dis [Epub ahead of print]

Download references

Interessenkonflikt

Die korrespondierende Autorin weist auf folgende Beziehungen hin: Referententätigkeiten, Reisekostenübernahmen durch Roche, Chugai, Abbott.

Author information

Authors and Affiliations

  1. Poliklinik für Rheumatologie, UKSH, Campus Lübeck, Klinikum Bad Bramstedt, Oskar-Alexander Str. 26, 24576, Bad Bramstedt, Deutschland

    J.U. Holle

Authors
  1. J.U. Holle
    View author publications

    You can also search for this author in PubMed Google Scholar

Corresponding author

Correspondence to J.U. Holle.

Rights and permissions

Reprints and permissions

About this article

Cite this article

Holle, J. ANCA („anti-neutrophil cytoplasm antibody“)-assoziierte Vaskulitiden. Z. Rheumatol. 72, 445–456 (2013). https://doi.org/10.1007/s00393-013-1211-0

Download citation

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s00393-013-1211-0

Schlüsselwörter

Keywords

Search

Navigation