Review Article | Published:

Treatment of ANCA-associated vasculitis

Nature Reviews Nephrology volume 10, pages 2536 (2014) | Download Citation

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.

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

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Affiliations

  1. Medizinische Klinik und Poliklinik IV, Nephrologisches Zentrum, University Hospital Munich, Campus Grosshadern, Ludwig-Maximilians-University, Marchioninstrasse 15, Munich D-81377, Germany.

    • Ulf Schönermarck
  2. University Hospital Schleswig-Holstein, Campus Lübeck, Ratzeburger Allee 160, Lübeck D-23538, Germany.

    • Wolfgang L. Gross
  3. IIIrd Medical Department, Klinikum Offenbach GmbH, Starkenburgring 66, KfH Renal Center Starkenburgring 70, Offenbach/Main D-63069, Germany.

    • Kirsten de Groot

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Contributions

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.

Competing interests

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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Correspondence to Kirsten de Groot.

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https://doi.org/10.1038/nrneph.2013.225

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