title: Sequential development of systemic vasculitis with anti-neutrophil cytoplasmic antibodies complicating anti-glomerular basement membrane disease.
abstract: Anti-neutrophil cytoplasmic antibodies (cANCA) were detected in 3 patients with anti-glomerular basement membrane (anti-GBM) disease. All 3 cases presented late in their clinical course, with severe renal involvement and alveolar hemorrhage. Anti-neutrophil cytoplasmic antibodies were associated with clinical features outwith the lungs and kidneys; in one case cANCA were initially absent but subsequently developed concurrently with the clinical appearance of systemic vasculitis as the anti-GBM antibody titer was falling. These findings confirm that cANCA can complicate anti-GBM disease and suggest that cANCA may identify a distinct subset of patients with anti-GBM disease, supporting a pathogenic role for cANCA in the development of systemic vasculitis.
authors.affiliation: Department of Renal Medicine, Manchester Royal Infirmary, UK.
authors.forename: D J
authors.initials: DJ
authors.lastname: O'Donoghue
authors.forename: C D
authors.initials: CD
authors.lastname: Short
authors.forename: P E
authors.initials: PE
authors.lastname: Brenchley
authors.forename: W
authors.initials: W
authors.lastname: Lawler
authors.forename: F W
authors.initials: FW
authors.lastname: Ballardie
mesh_terms: D019867:Anti-Glomerular Basement Membrane Disease / Q000276:immunology*; D001323:Autoantibodies / Q000032:analysis*; D001485:Basement Membrane / Q000276:immunology; D003593:Cytoplasm / Q000276:immunology; D004797:Enzyme-Linked Immunosorbent Assay; D005260:Female; D006801:Humans; D007678:Kidney Glomerulus / Q000276:immunology*; D008875:Middle Aged; D009504:Neutrophils / Q000276:immunology*; D011446:Prospective Studies; D011863:Radioimmunoassay; D014657:Vasculitis / Q000209:etiology / Q000276:immunology*
chemical_list: D001323:Autoantibodies