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Focal Segmental
Glomerulosclerosis
Venkatesh Karthikeyan,
Velammal Medical College
Focal Segmental
Glomerulosclerosis
Focal – Less than 50% glomeruli is affected
Segmental – involving a portion of glomerular tuft
Sclerosis – abnormal hardening
Commonest cause of nephrotic sydrome
Causes
Idiopathic
Secondary
Loss of renal tissue (unilateral renal agenesis,
advanced stages of reflux nephropathy,
hypertensive nephropathy)
Associated with conditions like sickle cell
anemia, HIV, Heroin abuse, obesity
Mutations in genes like nephrin, podocin(ARD),
alpha – actinin 4(ADD)
Hallmark feature
 Degeneration and focal disruption of visceral epithelial cells
Light Microscopy
Sclerosis (extracellular accumulation of
collagenous matrix in mesangium or capillary
loops or both)
Hyalinosis (extracellular accumulation of
homogenous and eosinophilic material in
glomeruli)
Electron Microscopy
 Diffuse effacement of foot process of podocytes
Immunofluorescence microscopy
 IgM and C3 may be seen in the sclerotic areas and in
mesangium
Clinical Features
 Non-selective proteinuria
 Reduction in GFR
 Hypertension
 Poor response to corticosteroids
 Recurrence common in transplant patients
 Children have better prognosis
Collapsing Glomerulopathy
 Variant of FSG
 Usually associated with HIV and cystinosis
 Poor prognosis
 Collapse and sclerosis of the entire glomerular tuft
Other variants of FSG
 Glomerular tip lesion variant
 Cellular variant
 Perihilar variant
 Not otherwise specific (NOS) variant
Take home points
 Hallmark of FSG is degeneration and focal disruption of
visceral epithelial cells
 Hyalinosis and sclerosis seen
 Commonest cause for nephrotic syndrome
 Collapsing glomerulopathy is a variant of FSG seen in HIV
patients
 No Immunoglobulin or complement deposits seen on
immunofluorescence microscopy in Minimal change disease
unlike FSG
Thank you 
Dr Richard Bright
Father of Nephrology
Dr KS Chugh
Father of Nephrology in India

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Focal Segmental Glomerulosclerosis - Pathology - FSGS

Editor's Notes

  1. Rudolf virchow, richard bright
  2. – representing leaked plasma proteins due to endothelial or capillary wall injury – hyalinization and sclerosis occludes capillary lumen and forms fibrous adhesion between sclerotic portions of glomoeruli and nearby parietal epithelium
  3. Thinning or shortening
  4. Lysosomal storage disorder characterised by abnormal accumulation of cystine