Rapidly progressive glomerulonephritis other diagnostic studies

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Overview

The sensitivity and the specificity of ANCA testing for pauci-immune glomerulonephritis is only 80-90%, a renal biopsy is crucial for diagnosis of rapidly progressive glomerulonephritis. It helps to determine the severity of the disease. The initiation of therapy should not be delayed for biopsy results. Renal biopsy specimens show a diffuse, proliferation, necrotizing glomerulonephritis with crescent formation. Immunofluorescence microscopy shows finding of linear deposition of immunoglobulin G (IgG) along the glomerular capillaries and occasionally the distal tubules.

Renal biopsy

Light microscopy usually shows[1]

  • In microscopic polyangitis and Wegener’s granulomatosis: crescentic glomerulonephritis can present without fibrinoid necrosis.
  • Focal and segmental mesangial proliferative glomerulonephritis.
  • Necrotizing glomerulonephritis, focal proliferative and membranous with foci of fibrinoid necrosis were the next most common category having 1 case each..
  • Tubular changes in the form of atrophy and presence of casts and glomerular sclerosis (73.33%) were the next most common changes followed by peri-glomerular infiltrate along with myointimal hyperplasia
  • Glomerular basement membrane thickening and arterial hyalinization
  • Tubular atrophy and focal interstitial infiltration
  • Fibrinoid necrosis of the glomerular tuft was seen in 80% of the glomeruli.
  • Neutrophilic infiltration of the glomerular capillary loops.
  • 45% of glomeruli had (predominantly cellular) crescents and 23% were globally sclerotic.
  • Increased mesangial proliferation
  • Interstitial edema: interstitial oedema was seen in 80% of ANCA positive biopsies and interstitial infiltrate was seen either focally or in diffuse fashion.
  • Focal tubular epithelial flattening as the most common findings seen in tubules of pauci-immune glomerulonephritis.
  • In interstitium; interstitial infiltration by leukocytes is common and is most pronounced adjacent to severely inflamed glomeruli or vessels.
  • interstitial eosinophilic infiltrate

Immunofluorescence microscopy

References

  1. Minz RW, Chhabra S, Joshi K, Rani L, Sharma N, Sakhuja V, Duggal R, Pasricha N (2012). "Renal histology in pauci-immune rapidly progressive glomerulonephritis: 8-year retrospective study". Indian J Pathol Microbiol. 55 (1): 28–32. doi:10.4103/0377-4929.94850. PMID 22499296.

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