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  • Review Article
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Therapeutics of Wegener's granulomatosis

Nature Clinical Practice Rheumatology volume 2, pages 192–200 (2006)Cite this article

Abstract

The treatment of Wegener's granulomatosis, one of the most common forms of systemic vasculitis, has changed substantially over the past two decades. The principal aims of therapy are to control the disease swiftly, to limit the extent and severity of permanent organ damage, and to minimize the short-term and long-term morbidities that often result from therapy. This review provides an overview of the treatment regimens that are currently available for inducing and maintaining remission in patients with Wegener's granulomatosis, and also discusses newer agents that might have a role in the management of this disease in the future. Severe toxicity associated with the available agents and, therefore, there is keen interest in the development of alternative treatment strategies for this disease.

Key Points

  • Remission induction in severe Wegener's granulomatosis requires cyclophosphamide and high doses of glucocorticoids

  • Standard first-line remission-maintenance therapy consists of either methotrexate or azathioprine

  • The combination of methotrexate and glucocorticoids induces remission in most patients with 'limited' Wegener's granulomatosis, thus sparing patients the potential adverse effects of cyclophosphamide

  • In contrast to its efficacy in the treatment of some other systemic inflammatory conditions associated with autoimmunity, the tumor necrosis factor inhibitor etanercept does not seem to be effective in Wegener's granulomatosis

  • All patients with Wegener's granulomatosis should receive prophylaxis against Pneumocystis jiroveci pneumonia

  • Other concomitant therapies are important for the prevention of potential treatment-related toxicities

  • In the setting of treatment-refractory disease, several investigational approaches are possible

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Figure 1: Treatment algorithm for patients with Wegener's granulomatosis.

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Acknowledgements

JH Stone is a Hugh and Renna Cosner Scholar in the Center for Innovative Medicine at Johns Hopkins University.

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Authors and Affiliations

  1. Department of Medicine, Senior Resident at the Johns Hopkins Bayview Medical Center, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    Peter K Wung

  2. Division of Rheumatology, Associate Professor of Medicine and Director of The Johns Hopkins Vasculitis Center, Johns Hopkins University School of Medicine, Baltimore, MD, USA

    John H Stone

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Correspondence to John H Stone.

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Wung, P., Stone, J. Therapeutics of Wegener's granulomatosis. Nat Rev Rheumatol 2, 192–200 (2006). https://doi.org/10.1038/ncprheum0139

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