The Complement System and ANCA Associated Vasculitis in the Era of Anti-Complement Drugs.

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  • Author(s): Kimoto Y;Kimoto Y; Horiuchi T; Horiuchi T
  • Source:
    Frontiers in immunology [Front Immunol] 2022 Jun 23; Vol. 13, pp. 926044. Date of Electronic Publication: 2022 Jun 23 (Print Publication: 2022).
  • Publication Type:
    Journal Article; Review; Research Support, Non-U.S. Gov't
  • Language:
    English
  • Additional Information
    • Source:
      Publisher: Frontiers Research Foundation] Country of Publication: Switzerland NLM ID: 101560960 Publication Model: eCollection Cited Medium: Internet ISSN: 1664-3224 (Electronic) Linking ISSN: 16643224 NLM ISO Abbreviation: Front Immunol Subsets: MEDLINE
    • Publication Information:
      Original Publication: [Lausanne : Frontiers Research Foundation]
    • Subject Terms:
      Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis* ; Antibodies, Antineutrophil Cytoplasmic*; Complement System Proteins/therapeutic use ; Humans ; Kidney/pathology ; Neutrophils
    • Abstract:
      ANCA (anti-neutrophil cytoplasmic antibody)-associated vasculitis (AAV) is the condition in which ANCA, as an autoantibody, is associated with the pathogenesis of vasculitis in small blood vessels, mainly in the ear, nose, throat, kidney, lung, and nerves. These diseases are important because they can be fatal due to renal failure and pulmonary hemorrhage if not promptly and appropriately treated. Recently accumulated evidence has shown that C5a produced by the complement alternative pathway primes neutrophils, which in turn activate the complement alternative pathway, leading to the pathogenesis of AAV. Avacopan (CCX168), a C5aR antagonist was shown to be effective against AAV, and it has been a novel therapeutic option, becoming a novel anti-complement drug to modulate inflammatory diseases.
      Competing Interests: The authors declare that the research was conducted in the absence of any commercial or financial relationships that could be construed as a potential conflict of interest.
      (Copyright © 2022 Kimoto and Horiuchi.)
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    • Contributed Indexing:
      Keywords: ANCA; C5a; alternative pathway; avacopan; complement
    • Accession Number:
      0 (Antibodies, Antineutrophil Cytoplasmic)
      9007-36-7 (Complement System Proteins)
    • Publication Date:
      Date Created: 20220711 Date Completed: 20220712 Latest Revision: 20220720
    • Publication Date:
      20231215
    • Accession Number:
      PMC9260009
    • Accession Number:
      10.3389/fimmu.2022.926044
    • Accession Number:
      35812453