ANCA-Associated Vasculitis: Core Curriculum 2020

Duvuru Geetha, J. Ashley Jefferson

Research output: Contribution to journalReview articlepeer-review


Antineutrophil cytoplasmic antibody (ANCA)-associated vasculitis (AAV) is a group of disorders characterized by inflammation and destruction of small- and medium-sized blood vessels and the presence of circulating ANCA. Clinical disease phenotypes include granulomatosis with polyangiitis, microscopic polyangiitis, eosinophilic granulomatosis with polyangiitis, and renal-limited vasculitis. Serologic classification of AAV into proteinase 3–ANCA disease and myeloperoxidase-ANCA disease correlates with a number of disease characteristics. AAV has a predilection for the kidney, with >75% of patients having renal involvement characterized by rapidly progressive glomerulonephritis. The cause and pathogenesis of AAV are multifactorial and influenced by genetics, environmental factors, and responses of the innate and adaptive immune system. Randomized controlled trials in the past 2 decades have refined the therapy of AAV and transformed AAV from a fatal disease to a chronic illness with relapsing course and associated morbidity. This article in AJKD's Core Curriculum in Nephrology series provides a detailed review of the epidemiology, pathogenesis, diagnosis, and advances in the management of AAV.

Original languageEnglish (US)
Pages (from-to)124-137
Number of pages14
JournalAmerican Journal of Kidney Diseases
Issue number1
StatePublished - Jan 2020


  • Vasculitis
  • anti-neutrophil cytoplasmic antibody (ANCA)
  • autoantibody
  • complement
  • cyclophosphamide
  • glomerulonephritis
  • granulomatosis with polyangiitis (GPA)
  • immunosuppression
  • microscopic polyangiitis (MPA)
  • review
  • rituximab

ASJC Scopus subject areas

  • Nephrology


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