Focal Segmental Glomerulosclerosis

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Summary1)Fundamentals of Pathology by Hussain Sattar

Focal Segmental Glomerulosclerosis (FSGS) is the most common cause of nephrotic syndrome.

Pathophysiology2)Fundamentals of Pathology by Hussain Sattar

Histology – Collapsed glomeruli affected with FSGS collapsed variety (Wikipedia)

Five mutually exclusive variants of focal segmental glomerulosclerosis may be distinguished by the pathologic findings seen on renal biopsy: collapsing variant, glomerular tip lesion variant, cellular variant, perihilar variant and not otherwise specified (NOS) variant. The collapsing variant is associated with higher rate of progression to end-stage renal disease, whereas glomerular tip lesion variant has low rate of progression to end-stage renal disease in most patients.

Cellular variant shows similar clinical presentation to collapsing and glomerular tip variant but has intermediate outcomes between these two variants. However, because collapsing and glomerular tip variant show overlapping pathologic features with cellular variant, this intermediate difference in clinical outcomes may reflect sampling bias in cases of cellular focal segmental glomerulosclerosis. The prognostic significance of perihilar and NOS variants has not yet been determined.

Causes3)Fundamentals of Pathology by Hussain Sattar

  • Usually Idiopathic but is associated with HIV, heroin use and sickle cell disease

Symptoms 4)First Aid for the Basic Sciences: Organ Systems by Tao Le

  • Nonselective Proteinuria
  • Hypertension
  • Mild Hematuria
  • Decreased Renal Function

Diagnosis 5)First Aid for the Basic Sciences: Organ Systems by Tao Le

  • Renal Biopsy – focal and segmental sclerosis

– Diagram of the comparison of normal glomerulus and one affected with FSGS (ePain Assist)

Treatment 6)First Aid for the Basic Sciences: Organ Systems by Tao Le 7)Fundamentals of Pathology by Hussain Sattar

  • Poor Response to Steroids
  • Cyclophosphamide
  • Cyclosporine

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