Pauci-immune necrotizing glomerulonephritis.
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.
Rutgers
Abraham
A
Department of Rheumatology en Clinical Immunology, University Medical Center Groningen, University of Groningen, 9700 RB Groningen, The Netherlands. a.rutgers@int.umcg.nl
Sanders
Jan S F
JS
Stegeman
Coen A
CA
Kallenberg
Cees G M
CG
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
D019268:Antibodies, Antineutrophil Cytoplasmic; D007166:Immunosuppressive Agents