Rapidly progressive glomerulonephritis : recent advances in pathogenesis, diagnosis, and therapy

✍ Scribed by Weber, M.

• Publisher: Springer-Verlag
• Year: 1993
• Tongue: English
• Weight: 509 KB
• Volume: 71
• Category: Article
• ISSN: 1432-1440
• DOI: 10.1007/bf00190330

✦ Synopsis

Rapidly progressive glomerulonephritis

-Crescents -Anti-nuclear cytoplasmic autoantibodies -Anti-glomerular basement membrane disease -Pathogenesis Rapidly-progressive glomerulonephritis is commonly understood as a condition in which patients develop renal failure within weeks or months. Its histological counterpart is crescentic glomerulonephritis. However, the definition is not satisfactory since other types of nephritis occasionally also lead to rapid deterioration of renal function, and renal biopsy in crescentic glomerulonephritis may show a wide range of pathological changes. Arbitrary definitions, such as crescents affecting more than 50% of the glomerulus in more than 50% of the glomeruli, are of some help, but biopsy sampling error must taken into account, the percentage of crescents may change considerably within several days, and outcome may be more related to the underlying disease.

On immunhistological grounds, rapidly progressive crescentic glomerulonephritis can be divided into (a) pauci-immune glomerulonephritis, (b) glomerulonephritis with linear IgG along the glomerular basement membrane (GBM), and (c) those forms with granular immunoglobulin deposition. Immunhistology thereby roughly reflects the underlying clinical disorders, with the great majority of patients having microscopic renal vasculitis including idiopathic rapidly progressive crescentic glomerulonephritis, anti-GBM antibody mediated glomerulonephritis, systemic lupus erythematosus