title: Crescentic glomerulonephritis: new aspects of pathogenesis.
abstract: This review provides a summary of recent advances in the understanding of crescentic glomerulonephritis, focusing on antineutrophil cytoplasm antibody (ANCA)-associated vasculitis and anti-glomerular basement membrane (anti-GBM) antibody disease. In ANCA-associated vasculitis (AAV), four main conceptual advances are discussed as follows: (1) evidence for the pathogenicity of ANCA, (2) molecular mimicry and the role of infection in AAV, (3) evidence for aberrant T-cell responses and T-cell regulation in AAV, and (4) advances in understanding of genetic predisposition to AAV. In relation to anti-GBM disease we discuss the following: (1) the nature of the Goodpasture autoantigens, (2) T-cell responses and regulation in anti-GBM disease, and (3) human leukocyte antigen and non-human leukocyte antigen genetic associations.
authors.affiliation: Department of Medicine, Imperial College London, Hammersmith Hospital, London, United Kingdom. r.tarzi@imperial.ac.uk
authors.forename: Ruth M
authors.initials: RM
authors.lastname: Tarzi
authors.forename: H Terence
authors.initials: HT
authors.lastname: Cook
authors.forename: Charles D
authors.initials: CD
authors.lastname: Pusey
mesh_terms: D000818:Animals; D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000209:etiology / Q000235:genetics; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000276:immunology; D001323:Autoantibodies / Q000276:immunology; D005921:Glomerulonephritis / Q000209:etiology* / Q000276:immunology; D006801:Humans; D008213:Lymphocyte Activation; D052119:Lysosomal-Associated Membrane Protein 2; D051907:Lysosomal Membrane Proteins / Q000276:immunology; D009195:Peroxidase / Q000276:immunology; D013601:T-Lymphocytes / Q000276:immunology
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D001323:Autoantibodies; C497811:LAMP2 protein, human; D052119:Lysosomal-Associated Membrane Protein 2; D051907:Lysosomal Membrane Proteins; C051951:antiglomerular basement membrane antibody; D009195:Peroxidase