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Alternative agents in the treatment of granulomatosis with polyangiitis and microscopic polyangiitis

Author
Ronald J Falk, MD
Section Editors
Richard J Glassock, MD, MACP
Gerald B Appel, MD
Deputy Editor
Albert Q Lam, MD

NEW TERMINOLOGY

In January 2011, the Boards of Directors of the American College of Rheumatology (ACR), the American Society of Nephrology (ASN), and the European League Against Rheumatism (EULAR) recommended that the name "Wegener's granulomatosis" be changed to "granulomatosis with polyangiitis," abbreviated as GPA [1-3]. This change reflects a plan to gradually shift from honorific eponyms to a disease-descriptive or etiology-based nomenclature.

INTRODUCTION

Granulomatosis with polyangiitis, which can be abbreviated as GPA, and microscopic polyangiitis (MPA) are related systemic vasculitides. Both are associated with antineutrophil cytoplasmic antibodies (ANCA), have similar features on renal histology (eg, a focal necrotizing, pauci-immune glomerulonephritis), and have similar outcomes. There are, however, several differences between these disorders. (See "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis", section on 'Clinical presentation'.)

Patients with GPA or MPA are initially treated with immunosuppressive therapy to induce remission, most often consisting of glucocorticoids in combination with either cyclophosphamide or rituximab. Patients who attain remission are then treated with azathioprine or methotrexate, which are less toxic than cyclophosphamide, and low-dose glucocorticoids to maintain the remission. The usual duration of maintenance therapy after stable remission has been induced is 12 to 18 months. More prolonged maintenance therapy should be considered in patients who have multiple disease relapses. (See "Initial immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis" and "Maintenance immunosuppressive therapy in granulomatosis with polyangiitis and microscopic polyangiitis".)

Relapses occur either on maintenance therapy or after maintenance therapy has been discontinued. Patients who have more than minor relapses while on maintenance therapy and relapses occurring in patients who are no longer on maintenance immunosuppression are usually treated with reinstitution of the initial induction regimen. (See "Identification and management of relapsing disease in granulomatosis with polyangiitis and microscopic polyangiitis".)

Other therapies that have been evaluated for the induction and maintenance of remission and the treatment of relapses in patients with GPA or MPA will be reviewed here. The clinical manifestations and diagnosis of these disorders, the treatment of cyclophosphamide-resistant disease, and patient and renal outcomes are discussed elsewhere. (See "Clinical manifestations and diagnosis of granulomatosis with polyangiitis and microscopic polyangiitis" and "Treatment-resistant granulomatosis with polyangiitis and microscopic polyangiitis" and "Prognosis in granulomatosis with polyangiitis and microscopic polyangiitis, and management of those who develop end-stage renal disease".)

            

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Literature review current through: Nov 2016. | This topic last updated: Mon Jan 12 00:00:00 GMT+00:00 2015.
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References
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  1. Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. Arthritis Rheum 2011; 63:863.
  2. Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. J Am Soc Nephrol 2011; 22:587.
  3. Falk RJ, Gross WL, Guillevin L, et al. Granulomatosis with polyangiitis (Wegener's): an alternative name for Wegener's granulomatosis. Ann Rheum Dis 2011; 70:704.
  4. Rhee EP, Laliberte KA, Niles JL. Rituximab as maintenance therapy for anti-neutrophil cytoplasmic antibody-associated vasculitis. Clin J Am Soc Nephrol 2010; 5:1394.
  5. Roubaud-Baudron C, Pagnoux C, Méaux-Ruault N, et al. Rituximab maintenance therapy for granulomatosis with polyangiitis and microscopic polyangiitis. J Rheumatol 2012; 39:125.
  6. Jayne D. Rituximab treatment for vasculitis. Clin J Am Soc Nephrol 2010; 5:1359.
  7. Tesfa D, Ajeganova S, Hägglund H, et al. Late-onset neutropenia following rituximab therapy in rheumatic diseases: association with B lymphocyte depletion and infections. Arthritis Rheum 2011; 63:2209.
  8. Terrier B, Krastinova E, Marie I, et al. Management of noninfectious mixed cryoglobulinemia vasculitis: data from 242 cases included in the CryoVas survey. Blood 2012; 119:5996.
  9. Stegeman CA, Tervaert JW, Sluiter WJ, et al. Association of chronic nasal carriage of Staphylococcus aureus and higher relapse rates in Wegener granulomatosis. Ann Intern Med 1994; 120:12.
  10. Stegeman CA, 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.
  11. Israel HL. Sulfamethoxazole-trimethoprim therapy for Wegener's granulomatosis. Arch Intern Med 1988; 148:2293.
  12. Valeriano-Marcet J, Spiera H. Treatment of Wegener's granulomatosis with sulfamethoxazole-trimethoprim. Arch Intern Med 1991; 151:1649.
  13. Hoffman GS. Immunosuppressive therapy is always required for the treatment of limited Wegener's granulomatosis. Sarcoidosis Vasc Diffuse Lung Dis 1996; 13:249.
  14. Reinhold-Keller E, De Groot K, Rudert H, et al. Response to trimethoprim/sulfamethoxazole in Wegener's granulomatosis depends on the phase of disease. QJM 1996; 89:15.
  15. Seo P, Stone JH. The antineutrophil cytoplasmic antibody-associated vasculitides. Am J Med 2004; 117:39.
  16. Nowack R, Göbel U, Klooker P, et al. Mycophenolate mofetil for maintenance therapy of Wegener's granulomatosis and microscopic polyangiitis: a pilot study in 11 patients with renal involvement. J Am Soc Nephrol 1999; 10:1965.
  17. Langford CA, Talar-Williams C, Sneller MC. Mycophenolate mofetil for remission maintenance in the treatment of Wegener's granulomatosis. Arthritis Rheum 2004; 51:278.
  18. Koukoulaki M, Jayne DR. Mycophenolate mofetil in anti-neutrophil cytoplasm antibodies-associated systemic vasculitis. Nephron Clin Pract 2006; 102:c100.
  19. Ibernon M, Poveda R, Vidaller A, et al. Mycophenolate mofetil in anti-MPO renal vasculitis: an alternative therapy in case of cyclophosphamide or azathioprine toxicity. Clin Nephrol 2008; 69:395.
  20. Iatrou C, Zerbala S, Revela I, et al. Mycophenolate mofetil as maintenance therapy in patients with vasculitis and renal involvement. Clin Nephrol 2009; 72:31.
  21. Stassen PM, Tervaert JW, Stegeman CA. Induction of remission in active anti-neutrophil cytoplasmic antibody-associated vasculitis with mycophenolate mofetil in patients who cannot be treated with cyclophosphamide. Ann Rheum Dis 2007; 66:798.
  22. Joy MS, Hogan SL, Jennette JC, et al. A pilot study using mycophenolate mofetil in relapsing or resistant ANCA small vessel vasculitis. Nephrol Dial Transplant 2005; 20:2725.
  23. Hiemstra TF, Walsh M, Mahr A, et al. Mycophenolate mofetil vs azathioprine for remission maintenance in antineutrophil cytoplasmic antibody-associated vasculitis: a randomized controlled trial. JAMA 2010; 304:2381.
  24. Haubitz M, Koch KM, Brunkhorst R. Cyclosporin for the prevention of disease reactivation in relapsing ANCA-associated vasculitis. Nephrol Dial Transplant 1998; 13:2074.
  25. Allen NB, Caldwell DS, Rice JR, McCallum RM. Cyclosporin A therapy for Wegener's granulomatosis. Adv Exp Med Biol 1993; 336:473.
  26. Kamesh L, Harper L, Savage CO. ANCA-positive vasculitis. J Am Soc Nephrol 2002; 13:1953.
  27. Wegener's Granulomatosis Etanercept Trial (WGET) Research Group. Etanercept plus standard therapy for Wegener's granulomatosis. N Engl J Med 2005; 352:351.
  28. Stone JH, Holbrook JT, Marriott MA, et al. Solid malignancies among patients in the Wegener's Granulomatosis Etanercept Trial. Arthritis Rheum 2006; 54:1608.