Pathology of Nephrtic Syndrome NS

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Pathology of Nephrtic Syndrome (NS)

• Dr Nor Hayati Othman
• Associate Professor and Head
• Department of pathology

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NS - symptoms

• Massive proteinuria of gt 3.5 G per day
• Hypoalbuminaemia - plasma albumin lt3 gm per dl
• Generalised edema
• Hyperlipidaemia
• Other minor symptoms and signs

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NS - pathogenesis

• Excessive permeability of plasma proteins -
  gtgtheavy proteinuria
• Depletion of plasma proteins , mainly albumin -
  hypoalbuminaemia

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NS - Pathogenesis - 2

• Liver compensates but not successful - gtgt
  reversed AG ratio
• Reduced albumin -gt decreased colloid oncotic
  pressure of the blood -gt oedema

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NS - Pathogenesis - 3

• ADH is stimulated - gtgt oedema
• Increased Lipoprotein synthesis and decreased
  catabolism by liver -gtgt Hyperlipidaemia (mainly
  VLDL and /or LDL)
• HDL is also lost in urine when heavy
  proteinuria occurs

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NS - Pathogenesis - 4

• Loss of body proteins (immunoglobulins
  /complement) -gt frequent infection
• Loss of anticoagulants antithrombin III ,
  antiplasmin - gt thrombotic and embolic
  phenomenon

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NS - histology
Protein droplets in tubular cells
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NS - urine examination
Fatty casts
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NS - Causes

• Children
• Minimal Change GN 65
• Membranous GN 5
• Membrano-prolifrative GN

• Adult
• Membranous GN 40
• Diabetes Glomerulosclerosis
• Amyloidosis
• SLE
• Others

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Minimal Change GN

• Young children
• Most common cause of NS in children
• Proteinuria selective - only low molecular wt.
  ie Albumin

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Minimal Change GN - histology
Within normal histology
Within normal histology
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Minimal Change GN - EM
abnormal
normal
Fusion of foot processes
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MEMBRANOUS GN (MGN)

• Major cause of NS in adults
• Characterised by presence of electron-dense
  immune deposits along the epithelial side of GBM(
  subepithelium)

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MGN - EM
normal
abnormal
Thickened glom capp wall
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Pathogenesis - MGN

• Believed to be chronic antigen -antibody mediated
  diseases
• Genetic - prevalence of HLR - DR/2 (Japanese) and
  HLR - DR/3 (European) patients
• ? imbalance of T - helper /T- suppressor cells
• Circulating immune complexes in 15 - 25 cases
• ? presence of intrinsic tissue antigen in the
  visceral epithelial cells

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MGN - causes

• Primary
• Secondary
• Diabetes
• SLE
• Amyloidosis
• infections malaria , HBV
• malignancy

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MGN - histology
Thickened capp loop
No cellular proliferation
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Normal histology - glomerulus