ANCA-associated vasculitis, adult

Patrick Nachman, Shannon L. Murphy

Research output: Chapter in Book/Report/Conference proceedingChapter

Abstract

Antineutrophil cytoplasmic antibody (ANCA) associated vasculitis is a pauci-immune systemic small vessel vasculitis. ANCA are autoantibodies directed toward an antigen found in neutrophils, either myeloperoxidase (MPO) or proteinase 3 (PR3). ANCA are pathogenic and cause disease by activating neutrophils, which damage blood vessels. ANCA vasculitis may affect various organs and is associated with glomerulonephritis in most patients. The disease can be classified into one of a few phenotypes: microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA, formerly Wegener's granulomatosis), and eosinophilic granulomatosis with polyangiitis (EGPA, formerly Churg-Strauss syndrome). However, recent evidence has shown that the ANCA antigen specificity (i.e., MPO- or PR3-ANCA) may be more important in characterizing the disease than the pathologic phenotype (MPA, GPA, or EGPA). ANCA vasculitis is organ- and lifethreatening in many cases, but with early and appropriate therapy, patients can remit. Therapies including corticosteroids, cyclophosphamide, rituximab, and plasmapheresis are effective in inducing a remission in the vast majority of patients. However, relapse is unfortunately common and the existing options for both induction and maintenance therapy are associated with risks for substantial adverse effects. The risks of continued immunosuppression with maintenance therapy must be weighed against the risk of relapsed disease, and there is currently no clear answer regarding the optimal duration of immunotherapy. Although our understanding of this disease has come a long way in a relatively short time, there is still much work to be done in understanding the pathophysiology of disease and identifying better treatment options.

Original languageEnglish (US)
Title of host publicationGlomerulonephritis
PublisherSpringer International Publishing
Pages333-348
Number of pages16
ISBN (Electronic)9783319493794
ISBN (Print)9783319493787
DOIs
StatePublished - Feb 26 2019

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Antineutrophil Cytoplasmic Antibodies
Vasculitis
Granulomatosis with Polyangiitis
Microscopic Polyangiitis
Myeloblastin
Peroxidase
Neutrophils
Churg-Strauss Syndrome
Phenotype
Antigens
Plasmapheresis
Antibody Specificity
Therapeutics
Glomerulonephritis
Secondary Prevention
Autoantibodies
Immunotherapy
Cyclophosphamide
Immunosuppression
Blood Vessels

Keywords

  • ANCA vasculitis
  • Eosinophilic granulomatosis with polyangiitis
  • Granulomatosis with polyangiitis
  • Microscopic polyangiitis
  • Pauci-immune necrotizing glomerulonephritis
  • Pulmonary-renal syndrome
  • Rapidly progressive glomerulonephritis
  • Systemic vasculitis

Cite this

Nachman, P., & Murphy, S. L. (2019). ANCA-associated vasculitis, adult. In Glomerulonephritis (pp. 333-348). Springer International Publishing. https://doi.org/10.1007/978-3-319-49379-4_17

ANCA-associated vasculitis, adult. / Nachman, Patrick; Murphy, Shannon L.

Glomerulonephritis. Springer International Publishing, 2019. p. 333-348.

Research output: Chapter in Book/Report/Conference proceedingChapter

Nachman, P & Murphy, SL 2019, ANCA-associated vasculitis, adult. in Glomerulonephritis. Springer International Publishing, pp. 333-348. https://doi.org/10.1007/978-3-319-49379-4_17
Nachman P, Murphy SL. ANCA-associated vasculitis, adult. In Glomerulonephritis. Springer International Publishing. 2019. p. 333-348 https://doi.org/10.1007/978-3-319-49379-4_17
Nachman, Patrick ; Murphy, Shannon L. / ANCA-associated vasculitis, adult. Glomerulonephritis. Springer International Publishing, 2019. pp. 333-348
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