Glomerulonephropathies Syndromes

GN syndrome 1

Number of gloms affected:

  • Diffuse: majority of glomeruli are abnormal
  • Focal: some glomeruli affected

 

Extent of which individual glom is affected:

  • Global: entire glomerulus is abnormal
  • Segmental: only part of the glomerulus is affected

 


Goodpasture’s Disease:

  • Antibodies against type IV collagen -> lungs + kidney (GBM)
  • 3rd + 6th decade, more in males
  • Presents as RPGN type 1 + hemoptysis\dyspnea + pulmonary hemorrhage
  • Rx: plasma exchange, cyclophosphamide, prednisone

 


Henoch–Schönlein Purpura:

  • Vasculitis, secondary to IgA, mainly in children
  • Palpable non-tender purpura on buttocks\legs, abdominal pain, arthralgia
  • Associated w\ IgA nephropathy (Berger ds)
  • Dx: biopsyGN syndrome 2

 


ANCA-associated Vasculitis:

1. Eosinophilic granulomatosis with polyangiitis (Churg-Strauss syndrome):

  • P-ANCA
  • Necrotizing small and medium vessel vasculitis + granulomatous + eosinophilia
  • Asthma and eosinophilia
  • RPGN type III: Pacui-immune GN
  • Dx: biopsy

GN syndrome 3

 

2. Microscopic polyangitis:GN syndrome 4

  • P-ANCA
  • Necrotizing small-vessel vasculitis
  • RPGN type III: Pacui-immune GN

 

3. Granulomatosis with polyangitis (Wegener):

  • C-ANCA
  • Necrotizing small and medium vessel vasculitis, associated w\ granulomas formation
    • Upper resp: sinusitis, nasal septum perforation, otitis media, conjunctivitis
    • Lower resp: granulomas -> hemoptysis, cough, SOB
    • Renal: RPGN type III: Pacui-immune GN
    • Skin nodules\purpura, arthralgia

 


Cryoglobulinemia:

  • Cryoglobulins: monoclonal IgM + polyclonal IgG -> precipitate at reduced temperatures
  • Associated w\ Hep C
  • Sx: fever, arthralgia, Raynaud’s, ↓ C4
  • RPGN type II

GN syndrome 8

 


Alport Syndrome:

  • Hereditary nephritis (X-linked) associated with hearing loss
  • Almost all have hematuria
  • Eye problems (lenticonus, kerataconus, cataracts)

 


Download the PDF version: here


References:

  • Step up to medicine
  • Toronto notes
  • Master the boards

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