ANCA vasculitis has an associated autoimmune response that produces ANCAs that induce distinct pathologic lesions. Pauci-immune necrotizing and crescentic GN is a frequent component of ANCA vasculitis. ANCA vasculitis is associated with ANCA specific for myeloperoxidase (MPO-ANCA) or proteinase 3 (PR3-ANCA). A diagnosis of ANCA vasculitis should always specify the serotype as MPO-ANCA positive, PR3-ANCA positive, or ANCA-negative. To fully characterize a patient, the serotype also should be accompanied by the clinicopathologic variant if this can be determined: microscopic polyangiitis, granulomatosis with polyangiitis (Wegener), eosinophilic granulomatosis with polyangiitis (Churg–Strauss), or renal-limited vasculitis. ANCA vasculitis is most prevalent in individuals >50 years old. There are racial/ethnic and geographic influences on the prevalence, serotype frequencies, and clinicopathologic phenotypes. There is clinical, in vitro, and animal model evidence that ANCAs cause disease by activating neutrophils to attack small vessels. Immunomodulatory and immunosuppressive therapies are used to induce remission, maintain remission, and treat relapses. Over recent years, there have been major advances in optimizing treatment by minimizing toxic therapy and utilizing more targeted therapy.
|Original language||English (US)|
|Number of pages||12|
|Journal||Clinical Journal of the American Society of Nephrology|
|State||Published - Oct 2017|
Bibliographical noteFunding Information:
J.C.J. is on the unbranded speaker bureau for Genentech. Some of this work was supported by federal grant P01 DK058335-06 from the National Institutes of Health/National Institute of Diabetes and Digestive and Kidney Diseases.
© 2017 by the American Society of Nephrology.
- Antineutrophil cytoplasmic
- Churg-strauss syndrome
- Granulomatosis with polyangiitis
- Microscopic polyangiitis
- Neutrophil activation