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Pathology of ANCA-associated vasculitis

  • Review Article
  • The Asia Pacific Meeting of Vasculitis and ANCA Workshop 2012
  • Published:
Clinical and Experimental Nephrology Aims and scope Submit manuscript

Abstract

According to the recent 2012 Chapel Hill Consensus Conference, ANCA-associated vasculitis is classified in the group of small vessel vasculitis. Pauci-immune necrotizing crescentic glomerulonephritis is the morphological hallmark of ANCA-associated vasculitis. Crescentic damage of the glomerular tuft is characterized by macrophage accumulation through vascular cell adhesion molecule-1 (VCAM-1) activation. Macrophages have a key role in chronic progression of renal damage due to production of substances involved in matrix remodelling [transforming growth factor-beta (TGFβ)]. Diffuse interstitial infiltration of T and B lymphocytes and macrophages is another frequent morphological feature. Tubulitis is considered an important marker of worse prognosis. Unfortunately, the renal changes on long-term follow-up are largely unknown due to the small number of studies with repeat biopsy in these disorders. Although a standardized score for renal biopsies was developed previously, a final histopathologic classification is still lacking. The European Vasculitis Study Group (EUVAS) proposed a classification system based on glomerular pathology as assessed by light microscopy.

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Authors and Affiliations

  1. Nephropathology Unit, Department of Pathology, San Gerardo Hospital, Università Milan Bicocca, 20900, Monza, Italy

    Franco Ferrario, Alice Vanzati & Fabio Pagni

Authors
  1. Franco Ferrario
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  2. Alice Vanzati
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  3. Fabio Pagni
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Correspondence to Franco Ferrario.

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Ferrario, F., Vanzati, A. & Pagni, F. Pathology of ANCA-associated vasculitis. Clin Exp Nephrol 17, 652–658 (2013). https://doi.org/10.1007/s10157-012-0701-8

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  • DOI: https://doi.org/10.1007/s10157-012-0701-8

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