Focal Segmental Glomerulosclerosis

Background and Epidemiology

  • Accounts for about 5-10% of nephrotic syndrome in children and about 40% in adults
  • Rare (estimated incidence of 7 per 1000000)
  • However, it is the most common primary glomerular disorder which causes end-stage renal failure.
  • Essentially progressive glomerular scarring

Aetiology and Types of FSGS

  • Secondary
    • I.e. to a pathological process e.g. sickle cell anaemia; HIV/Hep B infection; parvovirus B19 infection; inherited mutations of podocyte-related genes; immune-complex nephritis e.g. in SLE; drugs e.g. IV heroin, pamidronate, lithium; structural abnormalities
  • Primary (idiopathic) – most common, accounting for ~80% of cases

Pathology

  • Segmental areas of glomerular sclerosis with hyalinisation of glomerular capillaries and the segmental areas usually adherent to the Bowman’s capsule
    • Focal areas of tubular atrophy and interstitial nephritis are common
  • There may be ‘collapsing’ glomerulopathy (collapsed capillaries with basement membrane wrinkling and crowding of the epithelial cells)
    • Usually worse disease and prognosis

Clinical Presentation

Investigation

  • As nephrotic syndrome
    • NB Renal biopsy may appear normal depending on whether the biopsy is involved

Management

  • High dose steroids (60-80mg/day) can be trialed.
    • About half of patients show minimal/no response after 16 weeks
  • Cyclosporine may also be tried, but rarely cause complete remission if the patient showed no response to steroid therapy
  • Patients who do not respond to therapy, and those who have severe proteinuria at presentation, are more likely to deteriorate to end-stage kidney failure
    • Patients can also recur after transplantation
Advertisements

Leave a Reply

Fill in your details below or click an icon to log in:

WordPress.com Logo

You are commenting using your WordPress.com account. Log Out /  Change )

Google+ photo

You are commenting using your Google+ account. Log Out /  Change )

Twitter picture

You are commenting using your Twitter account. Log Out /  Change )

Facebook photo

You are commenting using your Facebook account. Log Out /  Change )

Connecting to %s