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When should patients with ANCA-associated vasculitis be switched from induction to maintenance therapy?

Nature Clinical Practice Nephrology volume 3, pages 236–237 (2007)Cite this article

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Wegener's granulomatosis and microscopic polyangiitis are the most important antineutrophil cytoplasm antibody (ANCA)-associated systemic vasculitides (AASV). AASV are characterized by the presence of necrotizing small-vessel vasculitis and circulating ANCAs. Since the introduction of daily oral cyclophosphamide and corticosteroids into the treatment of these formerly fatal diseases, remission is now achieved in over 90% of patients and the 5-year survival rate is about 80%.1,2 Improved diagnostic procedures, such as ANCA serology, imaging and immunohistology, have enabled earlier diagnosis of AASV and definition of different disease stages, and have greatly raised awareness of these diseases. Nevertheless, longer-term follow-up of patients treated with the empirical cyclophosphamide plus cortico steroid regimen for up to 3 years has demonstrated that AASV are associated with high morbidity rates resulting from chronic damage due to disease relapses1,2,3 and the toxic effects of treatment (e.g. lymphoma, bladder cancer, infertility and infections requiring hospitalization).

Concerns about the toxicity of cyclophosphamide in patients with AASV have inspired the design of protocols that either reduce the duration of cyclophosphamide treatment or completely avoid exposure to this agent.4 There is a general consensus that new or relapsing generalized vasculitis accompanied by threatened vital organ function must be controlled by a potent immunosuppressive regimen, consisting of a cytotoxic drug and prednisolone, for a limited period of time—usually 3–6 months. Once remission is achieved, a less myelosuppressive and less toxic regimen can be introduced to maintain remission and allow tapering of corticosteroids. The standard drugs used for maintenance therapy are azathioprine and methotrexate.5,6 Mycophenolate mofetil and leflunomide are alternatives; however, there is less published evidence to support their use.7

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Authors and Affiliations

  1. O Flossmann is a trainee in nephrology and general internal medicine at Addenbrooke's Hospital, Cambridge, UK,

    Oliver Flossmann

  2. K de Groot is Head of the Department of Nephrology/ Rheumatology at the Klinikum Offenbach GmbH, Offenbach am Main, Germany.,

    Kirsten de Groot

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  1. Oliver Flossmann
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  2. Kirsten de Groot
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Correspondence to Kirsten de Groot.

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Flossmann, O., de Groot, K. When should patients with ANCA-associated vasculitis be switched from induction to maintenance therapy?. Nat Rev Nephrol 3, 236–237 (2007). https://doi.org/10.1038/ncpneph0468

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