Rapidly progressive glomerulonephritis other diagnostic studies

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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mohammed Abdelwahed M.D[2]

Overview

Renal biopsy

  • The hallmark histologic lesions in most of the studies of acute pauci-immune ANCA positive as well as ANCA negative glomerulonephritis are documented as
  • crescents and fibrinoid tuft necrosis,
  • which occur at the same frequency irrespective of the presence or absence of associated systemic vasculitis [3,6,7] .
  • in microscopic polyangitis and Wegener’s granulomatosis observed necrotizing crescentic glomerulonephritis as the commonest histopathological diagnosis followed by crescentic glomerulonephritis without fibrinoid necrosis (23%), as the next most common histological diagnosis in patients with ANCA positive serology which almost corresponds to the work conducted by researchers in the western hemisphere. [8]
  • In the present study however, of all the ANCA positive cases on histology, the maximum number of cases were of ESRD accounting for 26.66% of cases. Focal and segmental mesangial proliferative and crescentic glomerulonephritis without fibrinoid necrosis were the next most common category (20.00%) followed by necrotizing crescentic glomerulonephritis (13.33%). Necrotizing glomerulonephritis, focal proliferative and membranous with foci of fibrinoid necrosis were the next most common category having 1 case each (6.66%). Thus in contrast to the documented finding of most common histological findings of cresenteric type glomerulonephritis in cases of systemic vasculitis by various researchers, diffuse global glomerulosclerosis/ ESRD was the most common histological finding in our group. The fact this was also the most common histological diagnosis in ANCA negative sub-group also speaks volume about the delayed presentation and patient ignorance as a great challenge to nephrologists in developing countries of Asia and Africa.
  • Of the 30 parameters under which every renal biopsy (120) in the present study was scored as detailed in materials and methods; the most common parameters observed in ANCA positive biopsies were- fibrinoid necrosis, glomerular loop neutrophil infiltration, interstitial oedema and arterial hyalinization (all seen in 80% ANCA positive renal biopsies). 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 accounted for 66.66%. On the other hand in ANCA negative non-pauciimmune glomerulonephritis cases the most common histological parameters were tubular casts (72.38%) followed by interstitial fibrosis (51.42%). Glomerular basement membrane thickening and arterial hyalinization (48.57%) and tubular atrophy and focal interstitial infiltration (44.76% in each) were other significant histological findings [Table/Fig-3].

Glomerular Changes

  • Of the ANCA positive biopsies crescents were noted in all the cases with fibrocellular being the commonest (40 %) followed equally by cellular and fibrous crescents (26.66%).
  • 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 glomerular basement membrane thickening
  • glomerulosclerosis
  • 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.
  • In both cases mononuclear infiltrate formed the predominant part (86.66%), of the infiltrate of ANCA positive cases followed by neutrophils and eosinophils in 26.66% and 20.00% cases respectively. Interstitial fibrosis was present in 60% of ANCA positive biopsies in this study. Many studies such as the one conducted by Haeur et al., have also noted the presence of interstitial oedema but only in 34% of biopsies along with interstitial infiltrates (predominantly mononuclear) in 92% of biopsies and fibrosis was present in 83% of biopsies.
  • interstitial eosinophilic infiltrate

References

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