Juvenile nasopharyngeal angiofibroma| aetiology, diagnosis, treatment

• 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.

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 : Here

Microscopic 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).

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