Pathogenesis and treatment of ANCA-associated vasculitis-a role for complement.
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.
Division of Nephrology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.|Department of Paediatrics, University of Toronto, Toronto, ON, Canada.
Damien
D
Noone
Division of Nephrology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada.|Department of Paediatrics, University of Toronto, Toronto, ON, Canada.
Diane
D
Hebert
Division of Nephrology, The Hospital for Sick Children, 555 University Avenue, Toronto, ON, M5G 1X8, Canada. christoph.licht@sickkids.ca.|Department of Paediatrics, University of Toronto, Toronto, ON, Canada. christoph.licht@sickkids.ca.|Program in Cell Biology, Research Institute, The Hospital for Sick Children, Toronto, ON, Canada. christoph.licht@sickkids.ca.|Department of Laboratory Medicine and Pathobiology, University of Toronto, Toronto, ON, Canada. christoph.licht@sickkids.ca.|Institute of Medical Science, University of Toronto, Toronto, ON, Canada. christoph.licht@sickkids.ca.
Christoph
http://orcid.org/0000-0003-4168-3277
C
Licht
D000818:Animals; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000503:physiopathology* / Q000628:therapy; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D003165:Complement System Proteins / Q000276:immunology*; D006801:Humans; D007155:Immunologic Factors / Q000627:therapeutic use; D010951:Plasma Exchange / Q000379:methods
D019268:Antibodies, Antineutrophil Cytoplasmic; D007155:Immunologic Factors; D003165:Complement System Proteins
Anti-neutrophil cytoplasm antibody; Children; Eosinophilic granulomatosis with polyangiitis; Granulomatosis with polyangiitis; Microscopic polyangiitis; treatment