• FSGS accounts for 10-15% of nephrotic syndrome in children.
• Adult can also be affected (15-200/0 of adult nephrotic syndrome).
• It is steroid resistant.
• It can lead to CRF within about 10 years.
• Secondary to :
- AIDS associated nephropathy.
- Heroin nephropathy.
- Reflux nephropathy.
- Bilharziasis.
- Obesity.
- Sickle cell disease.
- Solitary kidney.
• E.M :
- Focal = some glomeruli are affected.
- Segmental i.e part of each glomerulus is affected.
- Glomerulosclerosis(partial or total replacement of glomerulus
by hyaline material).
• Immunofluorescence: IgM - C3.
- First line, steroids (daily or every other day), prolonged therapy with taper for 6 months.
- Second line, for those with increased risks of steroids DM, obesity ==> cyclosporine or cyclophosphamide.
- Third line therapy: mycophenolate.
* Treatment of the cause in secondary FSGS.
* For both idiopathic or secondary forms: control blood pressure, ACE inhibitors, lipid control.
- Recurrence of FSGS in transplants occurs in 40% of patients.
- Heroin abusers-andpatients with HIV usually follow a much more rapid deterioration (progression to End-stage renal disease ESRD within < 1 year).
• Adult can also be affected (15-200/0 of adult nephrotic syndrome).
• It is steroid resistant.
• It can lead to CRF within about 10 years.
Causes of FSGS :
• Primary : idiopathic.• Secondary to :
- AIDS associated nephropathy.
- Heroin nephropathy.
- Reflux nephropathy.
- Bilharziasis.
- Obesity.
- Sickle cell disease.
- Solitary kidney.
Clinical picture of FSGS
Nephrotic syndrome : HereMicroscopic examination :
• Light: Almost normal.• E.M :
- Focal = some glomeruli are affected.
- Segmental i.e part of each glomerulus is affected.
- Glomerulosclerosis(partial or total replacement of glomerulus
by hyaline material).
• Immunofluorescence: IgM - C3.
Treatment of FSGS
* Immunosuppressive therapy for idiopathic FSGS:- First line, steroids (daily or every other day), prolonged therapy with taper for 6 months.
- Second line, for those with increased risks of steroids DM, obesity ==> cyclosporine or cyclophosphamide.
- Third line therapy: mycophenolate.
* Treatment of the cause in secondary FSGS.
* For both idiopathic or secondary forms: control blood pressure, ACE inhibitors, lipid control.
- Recurrence of FSGS in transplants occurs in 40% of patients.
- Heroin abusers-andpatients with HIV usually follow a much more rapid deterioration (progression to End-stage renal disease ESRD within < 1 year).