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Pathogenesis and treatment of ANCA-associated vasculitis—a role for complement

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Abstract

The antineutrophil cytoplasm autoantibody (ANCA)-associated vasculitides (AAV), although rare in childhood, can have devastating effects on affected organs, especially the kidney. In this review we present an update on the pathogenesis and treatment of ANCA vasculitis, with a particular emphasis on the role of the alternative pathway of complement. The rationale and evidence for the current treatment strategies are summarized. Targeting the activation of neutrophils by the anaphylatoxin C5a may serve as an additional therapeutic strategy, however the results of clinical studies are awaited.

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Authors and Affiliations

  1. Division of Nephrology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada

    Damien Noone, Diane Hebert & Christoph Licht

  2. Department of Paediatrics, University of Toronto, Toronto, ON, Canada

    Damien Noone, Diane Hebert & Christoph Licht

  3. Program in Cell Biology, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada

    Christoph Licht

  4. Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada

    Christoph Licht

  5. Institute of Medical Science, University of Toronto, Toronto, ON, Canada

    Christoph Licht

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  1. Damien Noone
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Correspondence to Christoph Licht.

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Noone, D., Hebert, D. & Licht, C. Pathogenesis and treatment of ANCA-associated vasculitis—a role for complement. Pediatr Nephrol 33, 1–11 (2018). https://doi.org/10.1007/s00467-016-3475-5

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  • DOI: https://doi.org/10.1007/s00467-016-3475-5

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