Focal segmental glomerulosclerosis is a nephrotic syndrome that is characterised by abnormal scarring (sclerosis) of some glomeruli (focal nature). The sclerosis affects only parts of the glomerulus (segmental nature).
It peaks between ages 20 and 30, and affects men more than women.
- Causes
- Idiopathic (primary)
- HIV
- IgA nephropathy
- Reflux nephropathy
- Heroin use
- Alport’s syndrome
- Sickle cell disease
- Pathophysiology
- Loss of podocytes → proliferation of mesangial and parietal epithelial cells → deposition of extracellular matrix in exposed areas → scar formation → chronic kidney disease
- Signs and symptoms
- Oedema
- Haematuria
- Hypertension
- Symptoms of chronic kidney disease
- Investigations
- Urinalysis
- Proteinuria > 3.5g/d
- Haematuria
- Urea an dcreatinine to estimate eGFR
- Hypercholesterolaemia
- Hypoalbuminaemia
- Blood glucose to exclude diabetic nephropathy
- Serology
- ANA and anti-dsDNA
- Complement levels
- ANCA
- HIV testing
- Hepatitis serology
- Serum and urine electrophoresis to exclude paraproteinaemia
- Haemoglobin electrophoresis if sickle cell disease is suspected
- Renal biopsy is the gold standard diagnostic test
- Focal and segmental sclerosis on light microscopy
- Urinalysis
- Treatment
- ACE inhibitors or ARBs to reduce proteinuria
- Statins for those with dyslipidaemia
- Diuretics for oedema
- Anticoagulation for those with increased risk of thromboembolism
- Corticosteroids are the first-line treatment in primary disease
- Calcineurin inhibitors are second-line
- Renal replacement therapy if indicated
- Plasma exchange and rituximab can be used for recurrence in renal transplant
- Complication