title: Pathology of ANCA-associated vasculitis.
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.
authors.affiliation: Nephropathology Unit, Department of Pathology, San Gerardo Hospital, Università Milan Bicocca, 20900, Monza, Italy. frferrario@gmail.com.
authors.forename: Franco
authors.initials: F
authors.lastname: Ferrario
authors.affiliation: Nephropathology Unit, Department of Pathology, San Gerardo Hospital, Università Milan Bicocca, 20900, Monza, Italy.
authors.forename: Alice
authors.initials: A
authors.lastname: Vanzati
authors.affiliation: Nephropathology Unit, Department of Pathology, San Gerardo Hospital, Università Milan Bicocca, 20900, Monza, Italy.
authors.forename: Fabio
authors.initials: F
authors.lastname: Pagni
mesh_terms: D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000150:complications / Q000276:immunology / Q000473:pathology*; D015415:Biomarkers / Q000032:analysis; D001706:Biopsy; D018450:Disease Progression; D005921:Glomerulonephritis / Q000276:immunology / Q000473:pathology*; D006801:Humans; D007150:Immunohistochemistry; D007678:Kidney Glomerulus / Q000276:immunology / Q000473:pathology*; D011237:Predictive Value of Tests; D011379:Prognosis; D012720:Severity of Illness Index
chemical_list: D015415:Biomarkers