title: Pauci-immune necrotizing glomerulonephritis.
abstract: Pauci-immune necrotizing glomerulonephritis is the most frequent cause of rapidly progressive glomerulonephritis and, in most cases, is associated with antineutrophil cytoplasmic antibodies (ANCA). It is either the renal manifestation of Wegener's granulomatosis, microscopic polyangiitis of Churg-Strauss syndrome, or a renal-limited vasculitis. In this review, the histopathologic changes seen in renal biopsies of patients with pauci-immune glomerulonephritis are described. The authors also describe why the disease is sometimes limited to the kidneys, the clinical course of renal disease, treatment issues, how to deal with disease relapses, and how to prevent them from occurring. Furthermore, the necessity of renal biopsy and rebiopsy, the usefulness of rapid ANCA detection at diagnosis, and serial measurement of ANCA during follow-up are discussed. The effect of dialysis on the disease process and the possibility of renal transplantation after disease remission are also debated.
authors.affiliation: Department of Rheumatology en Clinical Immunology, University Medical Center Groningen, University of Groningen, 9700 RB Groningen, The Netherlands. a.rutgers@int.umcg.nl
authors.forename: Abraham
authors.initials: A
authors.lastname: Rutgers
authors.forename: Jan S F
authors.initials: JS
authors.lastname: Sanders
authors.forename: Coen A
authors.initials: CA
authors.lastname: Stegeman
authors.forename: Cees G M
authors.initials: CG
authors.lastname: Kallenberg
mesh_terms: D056648:Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood* / Q000276:immunology; D001706:Biopsy; D005921:Glomerulonephritis / Q000276:immunology / Q000473:pathology* / Q000628:therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016030:Kidney Transplantation; D006435:Renal Dialysis
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents