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Treatment of ANCA-associated vasculitis

Key Points

  • Treatment of antineutrophil cytoplasmic autoantibody-associated vasculitides is tailored according to disease stage and severity

  • Current treatments for granulomatosis with polyangiitis and microscopic polyangiitis do not reflect the fact they are genetically distinct diseases

  • For remission induction, dose reduction and avoidance of prolonged use of cyclophosphamide have been successfully implemented; the addition of plasma exchange has increased the rate of renal recovery in patients with rapidly progressive glomerulonephritis

  • Maintenance of remission treatment to prevent relapse is even more important when reduced amounts of cyclophosphamide are used during induction of remission

  • Rituximab is noninferior to cyclophosphamide for remission induction, but its use as maintenance therapy is currently under investigation in randomized trials

  • In patients with life-threatening disease, severe renal involvement and/or alveolar haemorrhage, plasma exchange can be successfully used as adjunctive therapy

Abstract

Antineutrophil cytoplasmic autoantibody (ANCA)-associated diseases are small-vessel vasculitides, encompassing granulomatosis with polyangiitis (formerly Wegener's granulomatosis), microscopic polyangiitis and eosinophilic granulomatosis with polyangiitis. Once considered life-threatening diseases, the introduction of stage-adapted immunosuppressive therapy and medications with decreased toxicity has improved patients' survival. Treatment is biphasic, consisting of induction of remission (3–6 months) for rapid control of disease activity and maintenance of remission (at least 18 months) to prevent disease relapse using therapeutic alternatives that have reduced toxicity. This Review summarizes current treatment strategies for these diseases, with a special focus on long-term follow-up data from key randomized controlled trials and new developments in remission induction and maintenance therapy. Current treatment strategies have substantial short-term and long-term adverse effects, and relapses are frequent; thus, less-toxic and more-effective approaches are needed. Moreover, the optimal intensity and duration of maintenance therapy remains under debate. Clinical trials have traditionally considered ANCA-associated vasculitides as a single disease entity. However, future studies must stratify participants according to their specific disease, clinical features (different types of organ manifestation, PR3-ANCA or MPO-ANCA positivity) and disease severity.

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Figure 1: Treatment strategies for remission induction and maintenance of AAV.

References

  1. 1

    Jennette, J. C. et al. 2012 revised International Chapel Hill Consensus Conference nomenclature of vasculitides. Arthritis Rheum. 65, 1–11 (2013).

    CAS  PubMed  PubMed Central  Google Scholar 

  2. 2

    Jennette, C. J. & Falk, R. J. L1. Pathogenesis of ANCA-associated vasculitis: observations, theories and speculations. Presse Med. 42, 493–498 (2013).

    Google Scholar 

  3. 3

    Pepper, R. J. & Salama, A. D. Classifying and predicting outcomes in ANCA-associated glomerulonephritis. Nephrol. Dial. Transplant. 27, 2135–2137 (2012).

    PubMed  Google Scholar 

  4. 4

    Jayne, D. L27. Antibodies versus phenotypes: a clinician's view. Presse Med. 42, 579–582 (2013).

    PubMed  Google Scholar 

  5. 5

    Lyons, P. A. et al. Genetically distinct subsets within ANCA-associated vasculitis. N. Engl. J. Med. 367, 214–223 (2012).

    CAS  PubMed  PubMed Central  Google Scholar 

  6. 6

    Hoffman, G. S., Leavitt, R. Y., Kerr, G. S. & Fauci, A. S. The treatment of Wegener's granulomatosis with glucocorticoids and methotrexate. Arthritis Rheum. 35, 1322–1329 (1992).

    CAS  PubMed  Google Scholar 

  7. 7

    Slot, M. C., Tervaert, J. W., Boomsma, M. M. & Stegeman, C. A. Positive classic antineutrophil cytoplasmic antibody (C-ANCA) titer at switch to azathioprine therapy associated with relapse in proteinase 3-related vasculitis. Arthritis Rheum. 51, 269–273 (2004).

    CAS  PubMed  Google Scholar 

  8. 8

    Little, M. A. et al. Early mortality in systemic vasculitis: relative contribution of adverse events and active vasculitis. Ann. Rheum. Dis. 69, 1036–1043 (2010).

    PubMed  Google Scholar 

  9. 9

    Flossmann, O. et al. Long-term patient survival in ANCA-associated vasculitis. Ann. Rheum. Dis. 70, 488–494 (2011).

    Google Scholar 

  10. 10

    Wall, N. & Harper, L. Complications of long-term therapy for ANCA-associated systemic vasculitis. Nat. Rev. Nephrol. 8, 523–532 (2012).

    CAS  PubMed  Google Scholar 

  11. 11

    Holle, J. U. et al. Improved outcome in 445 patients with Wegener's granulomatosis in a German vasculitis center over four decades. Arthritis Rheum. 63, 257–266 (2011).

    Google Scholar 

  12. 12

    Hilhorst, M. et al. Improved outcome in anti-neutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis: a 30-year follow-up study. Nephrol. Dial. Transplant. 28, 373–379 (2013).

    CAS  PubMed  Google Scholar 

  13. 13

    Walton, E. W. Giant cell granuloma of the respiratory tract (Wegener's Granulomatosis). BMJ 2, 265–270 (1958).

    CAS  PubMed  Google Scholar 

  14. 14

    Hoffman, G. S. et al. Wegener's granulomatosis: an analysis of 158 patients. Ann. Intern. Med. 116, 488–498 (1992).

    CAS  PubMed  Google Scholar 

  15. 15

    Harper, L. et al. Pulse versus daily oral cyclophosphamide for induction of remission in ANCA-associated vasculitis: long-term follow-up. Ann. Rheum. Dis. 71, 955–960 (2012).

    CAS  PubMed  Google Scholar 

  16. 16

    de Groot, K. et al. Pulse versus daily oral cyclophosphamide for induction of remission in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized trial. Ann. Intern. Med. 150, 670–680 (2009).

    Google Scholar 

  17. 17

    Neumann, I. et al. Histological and clinical predictors of early and late renal outcome in ANCA-associated vasculitis. Nephrol. Dial. Transplant. 20, 96–104 (2005).

    PubMed  Google Scholar 

  18. 18

    de Groot, K. et al. Randomized trial of cyclophosphamide versus methotrexate for induction of remission in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 52, 2461–2469 (2005).

    CAS  PubMed  Google Scholar 

  19. 19

    Faurschou, M. et al. Brief report: long-term outcome of a randomized clinical trial comparing methotrexate to cyclophosphamide for remission induction in early systemic antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 64, 3472–3477 (2012).

    CAS  PubMed  Google Scholar 

  20. 20

    Faurschou, M. et al. Reply. Arthritis Rheum. 65, 844 (2013).

    PubMed  Google Scholar 

  21. 21

    Langford, C. A. & Hoffman, G. S. Methotrexate remains a valuable option for remission induction of nonsevere antineutrophil cytoplasmic antibody-associated vasculitis: comment on the article by Faurschou et al. Arthritis Rheum. 65, 843 (2013).

    CAS  PubMed  Google Scholar 

  22. 22

    Jones, R. B. A randomized trial of mycophenolate mofetil versus cyclophosphamide for remission induction of ANCA-associated vasculitis: “MYCYC”. On behalf of the European vasculitis study group [abstract]. Presse Med. 42, 678–679 (2013).

    Google Scholar 

  23. 23

    Casian, A. & Jayne, D. Plasma exchange in the treatment of Wegener's granulomatosis, microscopic polyangiitis, Churg-Strauss syndrome and renal limited vasculitis. Curr. Opin. Rheumatol. 23, 12–17 (2011).

    PubMed  Google Scholar 

  24. 24

    Jayne, D. R. et al. Randomized trial of plasma exchange or high-dosage methylprednisolone as adjunctive therapy for severe renal vasculitis. J. Am. Soc. Nephrol. 18, 2180–2188 (2007).

    CAS  Google Scholar 

  25. 25

    Walsh, M. et al. Long-term follow-up of patients with severe ANCA-associated vasculitis comparing plasma exchange to intravenous methylprednisolone treatment is unclear. Kidney Int. 84, 397–402 (2013).

    CAS  PubMed  Google Scholar 

  26. 26

    US National Library of Medicine. Clinicaltrials.gov [online], (2013).

  27. 27

    Pepper, R. J. et al. Intravenous cyclophosphamide and plasmapheresis in dialysis-dependent ANCA-associated vasculitis. Clin. J. Am. Soc. Nephrol. 8, 219–224 (2013).

    CAS  PubMed  Google Scholar 

  28. 28

    Szpirt, W. M., Heaf, J. G. & Petersen, J. Plasma exchange for induction and cyclosporine A for maintenance of remission in Wegener's granulomatosis—a clinical randomized controlled trial. Nephrol. Dial. Transplant. 26, 206–213 (2011).

    CAS  PubMed  Google Scholar 

  29. 29

    Walsh, M. et al. Plasma exchange for renal vasculitis and idiopathic rapidly progressive glomerulonephritis: a meta-analysis. Am. J. Kidney Dis. 57, 566–574 (2011).

    PubMed  Google Scholar 

  30. 30

    Casian, A. & Jayne, D. Management of alveolar hemorrhage in lung vasculitides. Semin. Respir. Crit. Care Med. 32, 335–345 (2011).

    PubMed  Google Scholar 

  31. 31

    Hruskova, et al. Long-term outcome of severe alveolar haemorrhage in ANCA-associated vasculitis: a retrospective cohort study. Scand. J. Rheumatol. 42, 211–214 (2013).

    CAS  PubMed  Google Scholar 

  32. 32

    Jones, R. B. et al. Rituximab versus cyclophosphamide in ANCA-associated renal vasculitis. N. Engl. J. Med. 363, 211–220 (2010).

    CAS  PubMed  PubMed Central  Google Scholar 

  33. 33

    Stone, J. H. et al. Rituximab versus cyclophosphamide for ANCA-associated vasculitis. N. Engl. J. Med. 363, 221–232 (2010).

    CAS  PubMed  PubMed Central  Google Scholar 

  34. 34

    Jones, R. B., Walsh, M. & Jayne, D. R. Two year follow up results from a randomised trial of RTX versus CyP for ANCA-associated vasculitis: RITUXVAS [abstract]. Clin. Exp. Immunol. 164, 57 (2011).

    Google Scholar 

  35. 35

    Specks, U. et al. Efficacy of remission-induction regimens for ANCA-associated vasculitis. N. Engl. J. Med. 369, 417–427 (2013).

    CAS  PubMed  PubMed Central  Google Scholar 

  36. 36

    Miloslavsky, E. M. et al. Clinical outcomes of remission induction therapy for severe antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 65, 2441–2449 (2013).

    CAS  PubMed  PubMed Central  Google Scholar 

  37. 37

    Clain, J. M. & Specks, U. S1. Rituximab for ANCA-associated vasculitis: the experience in the United States. Presse Med. 42, 530–532 (2013).

    PubMed  Google Scholar 

  38. 38

    Walsh, M., Merkel, P. A., Mahr, A. & Jayne, D. The effects of duration of glucocorticoid therapy on relapse rate in anti-neutrophil cytoplasm antibody associated vasculitis: a meta-analysis. Arthritis Care Res. 62, 1166–1173 (2010).

    Google Scholar 

  39. 39

    Cohen Tervaert, J. W. Rituximab in ANCA-associated vasculitis: a revolution? Nephrol. Dial. Transplant. 26, 3077–3079 (2011).

    PubMed  Google Scholar 

  40. 40

    Mansfield, N. et al. Prolonged disease-free remission following rituximab and low-dose cyclophosphamide therapy for renal ANCA-associated vasculitis. Nephrol. Dial. Transplant. 26, 3280–3286 (2011).

    CAS  PubMed  Google Scholar 

  41. 41

    Guerry, M. J. et al. Recommendations for the use of rituximab in anti-neutrophil cytoplasm antibody-associated vasculitis. Rheumatology (Oxford) 51, 634–643 (2012).

    CAS  Google Scholar 

  42. 42

    Jones, R. B. et al. A multicenter survey of rituximab therapy for refractory antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 60, 2156–2168 (2009).

    CAS  PubMed  Google Scholar 

  43. 43

    Martinez Del Pero, M. et al. B-cell depletion with rituximab for refractory head and neck Wegener's granulomatosis: a cohort study. Clin. Otolaryngol. 34, 328–335 (2009).

    CAS  PubMed  Google Scholar 

  44. 44

    Hamour, S. M. & Salama, A. D. ANCA comes of age-but with caveats. Kidney Int. 79, 699–701 (2011).

    PubMed  Google Scholar 

  45. 45

    Bomback, A. S. et al. ANCA-associated glomerulonephritis in the very elderly. Kidney Int. 79, 757–764 (2011).

    PubMed  Google Scholar 

  46. 46

    Pagnoux, C. et al. Treatment of systemic necrotizing vasculitides in patients >65 years old: results of the multicentre randomized CORTAGE trial [abstract]. Presse Med. 42, 679–680 (2013).

    Google Scholar 

  47. 47

    Holle, J. U. et al. 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 (2010).

    PubMed  Google Scholar 

  48. 48

    Pagnoux, C. et al. Wegener's granulomatosis strictly and persistently localized to one organ is rare: assessment of 16 patients from the French Vasculitis Study Group database. J. Rheumatol. 38, 475–478 (2011).

    PubMed  Google Scholar 

  49. 49

    DeRemee, R. A., McDonald, T. J. & Weiland, L. H. Wegener's granulomatosis: observations on treatment with antimicrobial agents. Mayo Clin. Proc. 60, 27–32 (1985).

    CAS  PubMed  Google Scholar 

  50. 50

    DeRemee, R. A. The treatment of Wegener's granulomatosis with trimethoprim/sulfamethoxazole: illusion or vision? Arthritis Rheum. 31, 1068–1074 (1988).

    CAS  PubMed  Google Scholar 

  51. 51

    Reinhold-Keller, E. et al. Response to trimethroprim-sulfamethoxazole (T/S) in Wegener's granulomatosis (WG) depends on the phase of disease. QJM 89, 15–23 (1996).

    CAS  PubMed  Google Scholar 

  52. 52

    Kallenberg, C. G. & Tadema, H. Vasculitis and infections: contribution to the issue of autoimmunity reviews devoted to “autoimmunity and infection”. Autoimmun. Rev. 8, 29–32 (2008).

    PubMed  Google Scholar 

  53. 53

    Hellmich, B. et al. 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 (2007).

    CAS  PubMed  Google Scholar 

  54. 54

    Rutgers, A. & Kallenberg, C. G. Refractory disease in antineutrophil cytoplasmic antibodies associated vasculitis. Curr. Opin. Rheumatol. 24, 245–251 (2012).

    PubMed  Google Scholar 

  55. 55

    Holle, J. U. et al. Rituximab for refractory granulomatosis with polyangiitis (Wegener's granulomatosis): comparison of efficacy in granulomatous versus vasculitic manifestations. Ann. Rheum. Dis. 71, 327–333 (2012).

    CAS  PubMed  Google Scholar 

  56. 56

    Jayne, D. R. et al. Intravenous immunoglobulin for ANCA-associated systemic vasculitis with persistent disease activity. QJM 93, 433–439 (2000).

    CAS  PubMed  Google Scholar 

  57. 57

    Martinez, V. et al. 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, 308–317 (2008).

    CAS  PubMed  Google Scholar 

  58. 58

    Mukhtyar, C. et al. Outcomes from studies of antineutrophil cytoplasm antibody associated vasculitis: a systematic review by the European League Against Rheumatism systemic vasculitis task force. Ann. Rheum. Dis. 67, 1004–1010 (2008).

    CAS  PubMed  Google Scholar 

  59. 59

    Booth, A. D. et al. Outcome of ANCA-associated renal vasculitis: a 5-year retrospective study. Am. J. Kidney Dis. 41, 776–784 (2003).

    PubMed  Google Scholar 

  60. 60

    Walsh, M. et al. Risk factors for relapse of antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 64, 542–548 (2012).

    CAS  PubMed  Google Scholar 

  61. 61

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

    CAS  Google Scholar 

  62. 62

    Lapraik, C. et al. BSR and BHPR guidelines for the management of adults with ANCA associated vasculitis. Rheumatology (Oxford) 46, 1615–1616 (2007).

    CAS  Google Scholar 

  63. 63

    Jayne, D. et al. A randomized trial of maintenance therapy for vasculitis associated with antineutrophil cytoplasmic autoantibodies. N. Engl. J. Med. 349, 36–44 (2003).

    CAS  Google Scholar 

  64. 64

    Pagnoux, C. et al. Azathioprine or methotrexate maintenance for ANCA-associated vasculitis. N. Engl. J. Med. 359, 2790–2803 (2008).

    CAS  Google Scholar 

  65. 65

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

    CAS  Google Scholar 

  66. 66

    WGET Research Group. Etanercept plus standard therapy for Wegener's granulomatosis. N. Engl. J. Med. 352, 351–361 (2005).

  67. 67

    Metzler, C. et al. Elevated relapse rate under oral methotrexate versus leflunomide for maintenance of remission in Wegener's granulomatosis. Rheumatology (Oxford) 46, 1087–1091 (2007).

    CAS  Google Scholar 

  68. 68

    European Vasculitis Study Group. Clinical Trial Protocol: REMAIN. [online], (2006).

  69. 69

    Little, M. A. et al. Therapeutic effect of anti-TNF-α antibodies in an experimental model of anti-neutrophil cytoplasm antibody-associated systemic vasculitis. J. Am. Soc. Nephrol. 17, 160–169 (2006).

    CAS  PubMed  Google Scholar 

  70. 70

    Cartin-Ceba, R. et al. Rituximab for remission induction and maintenance in refractory granulomatosis with polyangiitis (Wegener's): ten-year experience at a single center. Arthritis Rheum. 64, 3770–3778 (2012).

    CAS  Google Scholar 

  71. 71

    Rhee, E. P., Laliberte, K. A. & Niles, J. L. Rituximab as maintenance therapy for anti-neutrophil cytoplasmic antibody-associated vasculitis. Clin. J. Am. Soc. Nephrol. 5, 1394–1400 (2010).

    CAS  PubMed  PubMed Central  Google Scholar 

  72. 72

    Smith, R. M. et al. Rituximab for remission maintenance in relapsing antineutrophil cytoplasmic antibody-associated vasculitis. Arthritis Rheum. 64, 3760–3769 (2012).

    CAS  PubMed  Google Scholar 

  73. 73

    van Vollenhoven, R. F. et al. Longterm safety of patients receiving rituximab in rheumatoid arthritis clinical trials. J. Rheumatol. 37, 558–567 (2010).

    CAS  PubMed  Google Scholar 

  74. 74

    van Vollenhoven, R. F. et al. Long-term safety of rituximab in rheumatoid arthritis: 9.5-year follow-up of the global clinical trial programme with a focus on adverse events of interest in RA patients. Ann. Rheum. Dis. (2012).

  75. 75

    US National Library of Medicine. Clinicaltrials.gov [online], (2013).

  76. 76

    Charles, P. & Guillevin, L. S3. Rituximab for ANCA-associated vasculitides: the French experience. Presse Med. 42, 534–536 (2013).

    PubMed  Google Scholar 

  77. 77

    US National Library of Medicine. Clinicaltrials.gov [online], (2013).

  78. 78

    Stegeman, C. A., Cohen Tervaert, J. W., De Jong, P. E. & Kallenberg, C. G. Trimethoprim-sulfamethoxazole (co-trimoxazole) for the prevention of relapses of Wegener's granulomatosis. N. Engl. J. Med. 335, 16–20 (1996).

    CAS  PubMed  Google Scholar 

  79. 79

    Zycinska, K., Wardyn, K. A., Zielonka, T. M., Krupa, R. & Lukas, W. Co-trimoxazole and prevention of relapses of PR3-ANCA positive vasculitis with pulmonary involvement. Eur. J. Med. Res. 14 (Suppl. 4), 265–267 (2009).

    PubMed  PubMed Central  Google Scholar 

  80. 80

    Pagnoux, C. et al. Predictors of treatment resistance and relapse in antineutrophil cytoplasmic antibody-associated small-vessel vasculitis: comparison of two independent cohorts. Arthritis Rheum. 58, 2908–2918 (2008).

    PubMed  PubMed Central  Google Scholar 

  81. 81

    Pierrot-Deseilligny Despujol, C., Pouchot, J., Pagnoux, C., Coste, J. & Guillevin, L. Predictors at diagnosis of a first Wegener's granulomatosis relapse after obtaining complete remission. Rheumatology (Oxford) 49, 2181–2190 (2010).

    Google Scholar 

  82. 82

    Stegeman, A. C. et al. Association of chronic nasal carriage of staphylococcus aureus and higher relapse rates in Wegener's granulomatosis. Ann. Intern. Med. 120, 12–17 (1994).

    CAS  PubMed  Google Scholar 

  83. 83

    Boomsma, M. M. et al. Prediction of relapses in Wegener's granulomatosis by measurement of antineutrophil cytoplasmic antibody levels: a prospective study. Arthritis Rheum. 43, 2025–2033 (2000).

    CAS  PubMed  Google Scholar 

  84. 84

    Birck, R., Schmitt, W. H., Kaelsch, I. A. & van der Woude, F. J. Serial ANCA determinations for monitoring disease activity in patients with ANCA-associated vasculitis: systematic review. Am. J. Kidney Dis. 47, 15–23 (2006).

    PubMed  Google Scholar 

  85. 85

    Finkielman, J. D. et al. Antiproteinase 3 antineutrophil cytoplasmic antibodies and disease activity in Wegener granulomatosis. Ann. Intern. Med. 147, 611–619 (2007).

    PubMed  PubMed Central  Google Scholar 

  86. 86

    Tomasson, G., Grayson, P. C., Mahr, A. D., Lavalley, M. & Merkel, P. A. Value of ANCA measurements during remission to predict a relapse of ANCA-associated vasculitis—a meta-analysis. Rheumatology (Oxford) 51, 100–109 (2012).

    CAS  Google Scholar 

  87. 87

    Faurschou, M. et al. Malignancies in Wegener's granulomatosis: incidence and relation to cyclophosphamide therapy in a cohort of 293 patients. J. Rheumatol. 35, 100–105 (2008).

    CAS  Google Scholar 

  88. 88

    Lamprecht, P., et al. Effectiveness of TNF-alpha blockade with infliximab in refractory Wegener's granulomatosis. Rheumatology (Oxford) 41, 1303–1307 (2002).

    CAS  Google Scholar 

  89. 89

    Joy, M. S., Hogan, S. L., Jennette, J. C., Falk, R. J. & Nachman, P. H. A pilot study using mycophenolate mofetil in relapsing or resistant ANCA small vessel vasculitis. Nephrol. Dial. Transplant. 20, 2725–2732 (2005).

    CAS  PubMed  Google Scholar 

  90. 90

    Birck, R. et al. 15-Deoxyspergualin in patients with refractory ANCA-associated systemic vasculitis: a six-month open-label trial to evaluate safety and efficacy. J. Am. Soc. Nephrol. 14, 440–447 (2003).

    CAS  PubMed  Google Scholar 

  91. 91

    Schmitt, W. H., et al. Treatment of refractory Wegener's granulomatosis with antithymocyte globulin (ATG): an open study in 15 patients. Kidney Int. 65, 1440–1448 (2004).

    CAS  PubMed  Google Scholar 

  92. 92

    Metzler, C., Fink, C., Lamprecht, P., Gross, W. L. & Reinhold-Keller, E. Maintenance of remission with leflunomide in Wegener's granulomatosis. Rheumatology (Oxford) 43, 315–320 (2004).

    CAS  Google Scholar 

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U. Schönermarck and K. de Groot researched data for the article, made substantial contribution to discussion of the content, and wrote, reviewed and edited the manuscript before submission. W. L. Gross made a substantial contribution to discussion of the content and reviewed/edited the manuscript before submission.

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Correspondence to Kirsten de Groot.

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W. L. Gross declares that he has received honoraria for speaking and advisory board fees from Hoffmann-La Roche and GlaxoSmithKline. K. de Groot declares that she has received honoraria for speaking from Hoffmann–La Roche. U. Schönermarck declares no competing interests.

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Schönermarck, U., Gross, W. & de Groot, K. Treatment of ANCA-associated vasculitis. Nat Rev Nephrol 10, 25–36 (2014). https://doi.org/10.1038/nrneph.2013.225

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