Best Practice & Research Clinical Rheumatology
Volume 23, Issue 3 , Pages 367-378 , June 2009

Evidence-based management of ANCA vasculitis

  • David Carruthers, MBBS, PhD, FRCP (Consultant Rheumatologist)

      Affiliations

    • Department of Rheumatology, City Hospital, Sandwell and West Birmingham Hospitals NHS Trust, Birmingham, UK
    • Corresponding Author InformationCorresponding author. Tel.: +44 121 507 4853; Fax: +44 121 507 5451.
  • ,
  • Jonathan Sherlock, MB BChir, MRCP(UK) (Academic Clinical Fellow in Rheumatology)

      Affiliations

    • Sandwell and West Birmingham Hospitals NHS Trust, and Department of Rheumatology, Division of Immunity and Infection, University of Birmingham, Birmingham, UK

References 

  1. Lieb ES, Restivo C, Paulus HE. Immunosuppressive and corticosteroid therapy of polyarteritis nodosa. Am J Med. 1979;67:941–947
  2. Hoffman GS, Kerr GS, Leavitt RY, et al. Wegener granulomatosis - an analysis of 158 patients. Ann Intern Med. 1992;116:488–498
  3. Fauci AS, Wolff SM. Wegener's Granulomatosis: studies in eighteen patients and a review of the literature. Medicine. 1973;52:535–561
  4. Fauci AS, Haynes BF, Katz P, Wolff SM. Wegeners granulomatosis - prospective clinical and therapeutic experience with 85 patients for 21 years. Ann Intern Med. 1983;98:76–85
  5. Langford CA, Talar-Williams C, Barron KS, Sneller MC. A staged approach to the treatment of Wegener's granlomatosis. Arthritis Rheum. 1999;42:2666–2673
  6. Fries JF, Hunder GG, Bloch DA, et al. The American College of Rheumatology 1990 criteria for the classification of vasculitis - summary. Arthritis Rheum. 1990;33:1135–1136
  7. 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
  8. Jennette JC, Falk RJ, Andrassy K, et al. Nomenclature of systemic vasculitides - proposal of an international consensus conference. Arthritis Rheum. 1994;37:187–192
  9. Luqmani RA, Bacon PA, Moots RJ, et al. Birmingham Vasculitis Activity Score (BVAS) in systemic necrotizing vasculitis. QJM. 1994;87:671–678
  10. Exley AR, Bacon PA, Luqmani RA, et al. Development and initial validation of the vasculitis damage index for the standardized clinical assessment of damage in the systemic vasculitides. Arthritis Rheum. 1997;40:371–380
  11. Bacon PA, Moots RJ, Exley A, et al. VITAL assessment of vasculitis. Clin Exp Rheumatol. 1995;13:275–278
  12. Morgan MD, Harper L, Williams J, Savage C. Anti-neutrophil cytoplasm-associated vasculitis. J Am Soc Nephrol. 2006;17:1124–1134
  13. de Groot K, Rasmussen N, Bacon PA, et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52:2461–2469
  14. de Groot K, Muhler M, Reinhold Keller E, et al. Randomised controlled trial of daily oral versus pulsed cyclophosphamide for induction of remission in ANCA associated systemic vasculitis (abstract). Kidney Blood Pressure Res. 2005;28:195
  15. Jayne DRW, Gaskin G, Rasmussen N, et al. Randomized trial of plasma exchange or high dose methylprednisolone as adjunctive therapy for severe renal vasculitis. J Am Soc Nephrol. 2007;18:2180–2188
  16. Jayne D, Rasmussen N, Andrassy C, et al. A randomised trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic antibodies. N Engl J Med. 2003;349:36–44
  17. Gordon M, Luqmani RA, Adu D, et al. Relapses in patients with a systemic vasculitis. Q J Med. 1993;86:779–789
  18. Guillevin L, Cordier JF, Lhote F, et al. 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. 1997;40:2187–2198
  19. Haubitz M, Schellong S, Gobel U, et al. 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. 1998;41:1835–1844
  20. Reinhold-Keller E, Beuge N, Latza U, et al. An interdisciplinary approach to patients with Wegeners granulomatosis: long-term outcome in 155 patients. Arthritis Rheum. 2000;43:1021–1032
  21. The Wegener's Granulomatosis Etanercept Trial (WGET) Research Group . Etanercept plus standard therapy for Wegener's granulomatosis. The N Engl J Med. 2005;352:351–361
  22. de Groot K, Adu D, Savage COS. The value of pulse cyclophosphamide in ANCA-associated vasculitis: meta-analysis and critical review. Nephrol Dial Transplant. 2001;16:2018–2027
  23. de Groot K, Gross WL, Herlyn K, Reinhold-Keller E. Development and validation of a disease extent index for Wegener's granulomatosis. Clin Nephrol. 2001;55:31–38
  24. Hoffman GS, Leavitt RY, Kerr GS, Fauci AS. The treatment of Wegener's granulomatosis with glucocorticosteroids and methotrexate. Arthritis Rheum. 1992;35:1322–1329
  25. Sneller MC, Hoffman GS, Talar-Williams C, et al. An analysis of forty-two Wegener's granulomatosis patients treated with methotrexate and prednisone. Arthritis Rheum. 1995;38:608–613
  26. de Groot K, Muhler M, Reinhold-Keller E, et al. Induction of remission in Wegener's granulomatosis with low dose methotrexate. J Rheumatol. 1995;38:608–613
  27. Stone JH, Tun W, Hellman DB. Treatment of non-life threatening Wegener's granulomatosis with methotrexate and daily prednisone as the initial therapy of choice. J Rheumatol. 1999;26:1134–1139
  28. Guillevin L, Gasselin B, Cevallos R, et al. Microscopic polyangiitis - clinical and laboratory findings in eighty-five patients. Arthritis Rheum. 1999;42:421–430
  29. Hogan SL, Falk R, Chin H, et al. Predictors of relapse and treatment resistance in antineutrophil cyctopplasmic antibody-associated small-vessel vasculitis. Annals Intern Med. 2005;143:621–632
  30. Slot MC, Tervaert CJW, Boomsma MM, Stegeman CA. Positive classic antineutrophil cytoplasmic antibody (c-ANCA) titer at switch to azathioprine therapy associated with relapse in proteinase 3-related vasculitis. Arthritis Rheum. 2004;51:269–273
  31. Langford CA, Talar-Williams C, Barron KS, Sneller MC. Use of a cyclophosphamide-induction methotrexate-maintenance regimen for the treatment of Wegener's granulomatosis: extended follow-up and rate of relapse. Am J Med. 2003;114:463–469
  32. Specks U. Methotrexate for Wegener's granulomatosis: what is the evidence?. Arthritis Rheum. 2005;52:2237–2242
  33. de Groot K, Reinhold-Keller E, Tatsis E, et al. Therapy for the maintenance of remission in sixty-five patients with generalized Wegener's granulomatosis. Methotrexate versus trimethoprim/sulfamethoxazole. Arthritis Rheum. 1996;39:2052–2061
  34. Stegeman CA, Cohen Tervaert JW, de Jong PE, Kallenberg CG. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis. Dutch Co-Trimoxazole Wegener Study Group. N Engl J Med. 1996;335:16–20
  35. Lapraik C, Watts RW, Bacon P, et al. BSR and BHPR guidelines for the management of adults with ANCA-associated vasculitis. Rheumatology. 2007;46:1–11
  36. Mukhtyar C, Guillevin L, Cid M, et al. EULAR recommendations for the management of primary small and medium vessel vasculitis. Ann Rheum Dis. 2008;e publication: April 15
  37. Walton EW. Giant cell granuloma of the respiratory tract (Wegener's Granulomatosis). BMJ. 1957;2:265–270
  38. Mahr A, Pagnoux C, Cohen P. Treatment of ANCA associated vasculitides; corticosteroids and pulse cyclophopshamide followed by maintenance therapy with methotrexate or azathioprine a prospective multi-centre randomised trial (abstract). Kidney Blood Pressure Res. 2005;28:194
  39. Richter C, Schnabel A, Csernok E, et al. Treatment of antineutrophil cytoplasmic antibody (ANCA)-associated systemic vasculitis with high-dose intravenous immunoglobulin. Clin Exp Immunol. 1995;101:2–7
  40. Jayne DRW, Chapel H, Adu D, et al. Intravenous immunoglobulin for ANCA-associated systemic vascultis with persistent disease activity. QJM. 2000;93:433–439
  41. Schmitt WH, Hagen EC, Neumann I, et al. Treatment of refractory Wegener's granulomatosis with anti-thymocyte globulin (ATG): an open study in 15 patients. Kidney Int. 2004;65:1440–1448
  42. Keogh KA, Wylam ME, Stone JH, Specks U. Induction of Remission by B lymphocyte depletion in eleven patients with refractory antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 2005;52:262–268
  43. Lockwood CM, Thiru S, Stewart S, et al. Treatment of refractory Wegener's granulomatosis with humanized monoclonal antibodies. QJM. 1996;89:903–912

PII: S1521-6942(08)00153-8

doi: 10.1016/j.berh.2008.12.003

Best Practice & Research Clinical Rheumatology
Volume 23, Issue 3 , Pages 367-378 , June 2009