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

Damien Noone; Diane Hebert; Christoph Licht

doi:10.1007/s00467-016-3475-5

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

Pediatr Nephrol. 2018 Jan;33(1):1-11. doi: 10.1007/s00467-016-3475-5. Epub 2016 Sep 5.

Authors

Damien Noone^{1

2}, Diane Hebert^{1

2}, Christoph Licht^{3

4

5

6

7}

Affiliations

¹ Division of Nephrology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.
² Department of Paediatrics, University of Toronto, Toronto, ON, Canada.
³ Division of Nephrology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada. christoph.licht@sickkids.ca.
⁴ Department of Paediatrics, University of Toronto, Toronto, ON, Canada. christoph.licht@sickkids.ca.
⁵ Program in Cell Biology, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada. christoph.licht@sickkids.ca.
⁶ Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada. christoph.licht@sickkids.ca.
⁷ Institute of Medical Science, University of Toronto, Toronto, ON, Canada. christoph.licht@sickkids.ca.

PMID: 27596099
DOI: 10.1007/s00467-016-3475-5

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.

Keywords: Anti-neutrophil cytoplasm antibody; Children; Eosinophilic granulomatosis with polyangiitis; Granulomatosis with polyangiitis; Microscopic polyangiitis; treatment.

Publication types

Review

MeSH terms

Animals
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / complications
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / physiopathology*
Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / therapy
Antibodies, Antineutrophil Cytoplasmic / immunology
Complement System Proteins / immunology*
Humans
Immunologic Factors / therapeutic use
Plasma Exchange / methods

Substances

Antibodies, Antineutrophil Cytoplasmic
Immunologic Factors
Complement System Proteins