Focalproliferative Glomerulonephritis

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Renal pathology tutorial for nephrologists

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Focalproliferative Glomerulonephritis

  1. 1. Focal Proliferative GN
  2. 2. <ul><li>Focal proliferative pattern of glomerular injury consists of endocapillary or extracapillary (crescents) proliferative changes that occur in segmental or global fashion and involve less than 50% of all glomeruli. </li></ul><ul><li>The process can be pauci-immune, most commonly associated with ANCA. A number of immune complex-mediated processes can lead to this pattern of injury, such as lupus nephritis (class III) or IgA nephropathy. </li></ul>
  3. 3. <ul><li>Pauci-immune GN </li></ul><ul><li>Lupus Class III </li></ul><ul><li>IgA </li></ul>
  4. 4. <ul><li>Pauci-immune GN </li></ul><ul><ul><li>As in crescentic GN </li></ul></ul>
  5. 5. Lupus Nephritis Class III <ul><li>Histopathology: </li></ul><ul><li>Light microscopic examination reveals segmental or global endocapillary proliferative changes. The mesangium is usually expanded and hypercellular </li></ul><ul><li>The peripheral capillary loops are irregular in thickness, sometimes showing 'wire loops' and intraluminal 'microthrombi' (hyaline thrombi) </li></ul><ul><li>Leukocyte infiltration, focal necrosis, hematoxilin bodies, and cellular crescents can all be seen </li></ul><ul><li>In some cases, membranoproliferative pattern of injury may be dominant in glomeruli (class IV) </li></ul><ul><li>The tubulointerstitium may show active interstitial nephritis </li></ul><ul><li>Immunofluorescence: </li></ul><ul><li>There is 'full house' reactivity (reactivity for IgG, IgM, and IgA), with granular deposits in the mesangium. </li></ul>
  6. 6. <ul><li>Electron microscopy: </li></ul><ul><li>Visceral epithelial cells: Show different degrees of injury and degenerative changes, with focal, but sometimes extensive, effacement of foot processes. Subepithelial deposits can be seen in many cases </li></ul><ul><li>Glomerular basement membranes: May be irregular in thickness, with the presence of intramembranous, subepithelial, and/or subendothelial deposits. Subendothelial deposits can be rather large and may demonstrate substructural organization ('fingerprint'-like pattern) </li></ul><ul><li>Glomerular endothelial cells: May contain tubuloreticular structures </li></ul><ul><li>Mesangium: Expanded by increase in cellular elements and extracellular matrix, with sometimes large and confluent fine granular, electron-dense deposits </li></ul>
  7. 9. IgA <ul><li>Histopathology: </li></ul><ul><li>Light microscopic examination reveals mesangial hypercellularity and expansion of mesangial matrix, with segmental or global endocapillary proliferation and/or crescent formation </li></ul><ul><li>If the number of involved glomeruli is below 50 percent, it is designated as focal proliferative pattern; if there are 50 percent or more glomeruli involved, the process is designated as diffuse proliferative IgA glomerulonephritis </li></ul><ul><li>The tubulointerstitium may be unremarkable or show active inflammation or patchy fibrosis </li></ul><ul><li>Immunofluorescence: </li></ul><ul><li>There is dominant reactivity for IgA in the mesangium; C3 may be equally or less reactive. There is usually stronger reactivity for lambda than for kappa light chains in the mesangial deposits </li></ul>
  8. 10. <ul><li>Electron microscopy: </li></ul><ul><li>Visceral epithelial cells: Show different degrees of injury and degenerative changes; the effacement of foot processes is usually focal but sometimes extensive. Subepithelial and/or subendothelial deposits can also be present </li></ul><ul><li>Glomerular basement membranes: May be thin; there is a higher incidence of thin glomerular basement membrane disease in IgA nephropathy than in any other glomerular disease {6} </li></ul><ul><li>Glomerular endothelial cells: May show non-specific signs of injury; tubuloreticular structures are not seen </li></ul><ul><li>Mesangium: Shows increase in cellularity and extracellular matrix, with fine granular electron-dense deposits that are sometimes large and confluent </li></ul>

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