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ANCA-Associated Systemic Small-Vessel Vasculitis

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Primer on Nephrology

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Abstract

ANCA-associated vasculitides (AAV) are a group of clinicopathological syndromes, which include microscopic polyangiitis (MPA), granulomatosis with polyangiitis (GPA) and eosinophilic granulomatosis with polyangiitis (EGPA). These are characterised by necrotising small-vessel vasculitis with a relapsing and remitting course. AAV is a rare multisystem autoimmune disease, which results in rapidly progressive glomerulonephritis and immune-mediated destruction of other organs (e.g. the skin and lungs). Morbidity and mortality is a result of both the destructive, inflammatory disease process itself and of the high-intensity immunosuppression required to induce and maintain remission of this disease. The treatment regimen is guided by the severity of disease onset and multiple patient factors but is often based on use of high-dose corticosteroids and cyclophosphamide and/or rituximab.

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  1. Trinity Health Kidney Centre, Dublin, Ireland

    Jennifer Scott

  2. Tallaght University Hospital, Dublin, Ireland

    Mark A. Little

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    Mark Harber

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Scott, J., Little, M.A. (2022). ANCA-Associated Systemic Small-Vessel Vasculitis. In: Harber, M. (eds) Primer on Nephrology. Springer, Cham. https://doi.org/10.1007/978-3-030-76419-7_25

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