mindmap - Rapidly Progressive Glomerulonephritis

one of the more fatal renal manifestations - i pray that someday, a prophylaxis is found.

Rapidly Progressive GN

• general characteristics
  • clinical syndrome - not a specific etyologic form of GN
  • pathogenesis
    • those crescents are produced in part by proliferation of the parietal epithelial cells of Bowman's capsule in responce to injury and in part by infiltration of monocytes and macrophages
      caused by a number of different diseases
      • kidney
      • systemic
      most of the cases the injury is done by immunological pathways
      classification of 3
      • Anti-GBM 12%
        Immune complex 44%
        Pauci-immune (ANCA associated) 44%
• clinical features
  • Rapid and progressive loss of renal function with features of nephritic Syndrome
  • severe oliguria
  • may require long-term dyalysis / transplantation
  • death from renal failure within weeks / months
  • those with crescents in less then 80% of glomeruli have better prognosis
• characteristic histologic picture
  • presence of crescents
  • 
  • 
• Anti-GBM
  • characterised by
    • linear depots of IgG and in many cases, C3 on the GBM
      anti-GBM antibodies present in serum - helpful in diagnosis
      benefit from plasmapheresis - removal of pathogen from blood
  • causes
    • Idiopathic
    • Goodpasture
      • renal impairment with pulmonary involvement
      • clinical picture of pulmonary hemorrhages with renal failure
• Immune Complex
  • characteristics
    • immune-complex disorders
      can be a complication of any of
      the immune complex  nephritide
      • SLE
      • post-streptococcal
      • IgA nephropathy
      • henoch-scholein purpura
      granular pattern of staining of the GBM / mesangium
      cannot be helped with plasmapherisis
  • causes
    • Idiopathic
    • Henoch-Schonlein purpura
    • postinfectious
    • Hypersensitivity vasculitis
    • Cryoglobulinemia
    • SLE
• Pauci-immune
  • characteristics
    • defined by lack of anti - GBM antibodies
      or significant immune complex deposition
      most of them have antineutrophil cytoplasmic antibodies
      some cases are components of systemic vasculitis
      pauci-immune is by definition, limited to the kidney
  • causes
    • idiopathic
      • limited to kidney
    • Wegener's
    • Churg-Strauss
    • Microscopic Polyangitis
    • PAN