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: Abraham Rutgers, Jan S F Sanders, Coen A Stegeman, Cees G M Kallenberg
• Journal: Rheumatic diseases clinics of North America (Rheum Dis Clin North Am) Vol. 36 Issue 3 Pg. 559-72 (Aug 2010) ISSN: 1558-3163 [Electronic] United States
• PMID: 20688250 (Publication Type: Journal Article, Review)
• Copyright: Copyright 2010 Elsevier Inc. All rights reserved.

Chemical References

• Antibodies, Antineutrophil Cytoplasmic
• Immunosuppressive Agents

Topics

• Anti-Neutrophil Cytoplasmic Antibody-Associated Vasculitis (immunology, pathology, therapy)
• Antibodies, Antineutrophil Cytoplasmic (blood, immunology)
• Biopsy
• Glomerulonephritis (immunology, pathology, therapy)
• Humans
• Immunosuppressive Agents (therapeutic use)
• Kidney Transplantation
• Renal Dialysis