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ANCA Glomerulonephritis and Vasculitis.

J Charles Jennette1, Patrick H Nachman

  • 1Department of Pathology and Laboratory Medicine, Department of Medicine, and Kidney Center, School of Medicine, University of North Carolina at Chapel Hill, Chapel Hill, North Carolina.

Clinical Journal of the American Society of Nephrology : CJASN
|August 27, 2017
PubMed
Summary

Anti-neutrophil cytoplasmic antibody (ANCA) vasculitis involves an autoimmune response attacking small blood vessels. Diagnosis requires specifying the ANCA serotype (MPO-ANCA or PR3-ANCA) and clinicopathologic variant for effective treatment.

Keywords:
ANCAAnimalAntibodiesAntineutrophil CytoplasmicAutoantibodiesAutoimmunityChurg-Strauss SyndromeGranulomatosis with PolyangiitisMicroscopic PolyangiitisModelsMyeloblastinNeutrophil ActivationNeutrophilsPeroxidasePrevalenceRecurrenceSerogroupglomerulonephritisvasculitis

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Area of Science:

  • Rheumatology
  • Nephrology
  • Immunology

Background:

  • ANCA vasculitis is an autoimmune condition characterized by ANCA production.
  • Pauci-immune necrotizing and crescentic glomerulonephritis frequently occurs in ANCA vasculitis.
  • ANCAs are specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA).

Purpose of the Study:

  • To outline the diagnostic criteria for ANCA vasculitis.
  • To emphasize the importance of serotype and clinicopathologic variant determination.
  • To review current understanding of disease mechanisms and therapeutic advances.

Main Methods:

  • Review of clinical, in vitro, and animal model evidence.
  • Analysis of diagnostic classification including serotype and clinicopathologic variants.
  • Examination of immunomodulatory and immunosuppressive treatment strategies.

Main Results:

  • ANCA vasculitis diagnosis should specify MPO-ANCA, PR3-ANCA, or ANCA-negative status.
  • Clinicopathologic variants include microscopic polyangiitis, granulomatosis with polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited vasculitis.
  • Prevalence, serotype frequencies, and phenotypes are influenced by age, race, ethnicity, and geography.

Conclusions:

  • ANCAs activate neutrophils, leading to small vessel damage.
  • Effective management involves immunomodulatory and immunosuppressive therapies.
  • Recent advances focus on optimizing treatment with targeted therapies to minimize toxicity.